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Atrial Septal Defect (ASD)

Atrial Septal Defect (ASD)

Atrial septal defects (ASDs) are benign Benign Fibroadenoma acyanotic Acyanotic Tetralogy of Fallot congenital Congenital Chorioretinitis heart defects characterized by an opening in the interatrial septum that causes blood to flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure from the left atrium (LA) to the right atrium (RA) (left-to-right shunt). Atrial septal defects account for approximately 15% of all cases of congenital Congenital Chorioretinitis heart disease (CHD), making ASDs the 2nd most common CHD. There are 4 types of ASD ASD Autism spectrum disorder (ASD) is a neurodevelopmental disorder marked by poor social skills, restricted interests/social interactions, and repetitive/stereotyped behaviors. The condition is termed a "spectrum" because of the wide variability in the severity of symptoms exhibited. Autism Spectrum Disorder based on the location of the defect along the atrial septum, but the most common is the ostium secundum defect. Atrial septal defects are usually detected during a routine physical examination and confirmed by an echocardiogram Echocardiogram Transposition of the Great Vessels. In infancy, most small ASDs close spontaneously by 2 years of age. Only patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship that are symptomatic require surgical closure. In general, most patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship with ASD ASD Autism spectrum disorder (ASD) is a neurodevelopmental disorder marked by poor social skills, restricted interests/social interactions, and repetitive/stereotyped behaviors. The condition is termed a "spectrum" because of the wide variability in the severity of symptoms exhibited. Autism Spectrum Disorder can expect a good overall outcome.

Last updated: 14 Mar, 2022

Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

Contents

Overview

Definition

Atrial septal defect ( ASD ASD Autism spectrum disorder (ASD) is a neurodevelopmental disorder marked by poor social skills, restricted interests/social interactions, and repetitive/stereotyped behaviors. The condition is termed a “spectrum” because of the wide variability in the severity of symptoms exhibited. Autism Spectrum Disorder) is a communication Communication The exchange or transmission of ideas, attitudes, or beliefs between individuals or groups. Decision-making Capacity and Legal Competence between the right atrium (RA) and the left atrium (LA) secondary to a defect in the development of the atrial septum during embryogenesis. Atrial septal defect is usually a benign Benign Fibroadenoma condition and is classified as an acyanotic Acyanotic Tetralogy of Fallot congenital Congenital Chorioretinitis heart disease (CHD).

Classification

Atrial septal defects are classified based on the anatomic location of the defect:

  • Ostium secundum (75%–90% of cases): 
  • Ostium primum (15%):
    • Located on the lower part of the interatrial septum
    • Develops if the septum primum Septum primum Development of the Heart does not fuse with the endocardial cushion Endocardial cushion A fetal heart structure that is the bulging areas in the cardiac septum between the heart atria and the heart ventricles. During development, growth and fusion of endocardial cushions at midline forms the two atrioventricular canals, the sites for future tricuspid valve and bicuspid valve. Development of the Heart 
  • Sinus venosus (10%): 
    • Located in the upper and/or inferior part of the interatrial septum 
    • Caused by malposition of the insertion of the superior or inferior vena cavae 
  • Coronary sinus (1%): 
    • Not a true ASD ASD Autism spectrum disorder (ASD) is a neurodevelopmental disorder marked by poor social skills, restricted interests/social interactions, and repetitive/stereotyped behaviors. The condition is termed a “spectrum” because of the wide variability in the severity of symptoms exhibited. Autism Spectrum Disorder
    • The wall between the coronary sinus and the LA is missing.
Atrial septal defects locations

The anatomic locations of the different types of ASDs

Image by Lecturio.

Epidemiology

  • Account for 10%–15% of all cases of CHD
  • Incidence Incidence The number of new cases of a given disease during a given period in a specified population. It also is used for the rate at which new events occur in a defined population. It is differentiated from prevalence, which refers to all cases in the population at a given time. Measures of Disease Frequency: 0.67–2.1 per 1,000 live births
  • Girls > boys, with a girl-to-boy ratio of 2:1
  • The mortality Mortality All deaths reported in a given population. Measures of Health Status rate is low, at < 1%.

Etiology

  • Ostium secundum usually occurs as an isolated defect but may be associated with a syndrome (genetic component):
    • Holt-Oram syndrome: autosomal dominant Autosomal dominant Autosomal inheritance, both dominant and recessive, refers to the transmission of genes from the 22 autosomal chromosomes. Autosomal dominant diseases are expressed when only 1 copy of the dominant allele is inherited. Autosomal Recessive and Autosomal Dominant Inheritance, associated with upper limb deformity Deformity Examination of the Upper Limbs and heart block
    • Noonan’s syndrome: autosomal dominant Autosomal dominant Autosomal inheritance, both dominant and recessive, refers to the transmission of genes from the 22 autosomal chromosomes. Autosomal dominant diseases are expressed when only 1 copy of the dominant allele is inherited. Autosomal Recessive and Autosomal Dominant Inheritance, associated with facial defects, deformity Deformity Examination of the Upper Limbs of the sternum Sternum A long, narrow, and flat bone commonly known as breastbone occurring in the midsection of the anterior thoracic segment or chest region, which stabilizes the rib cage and serves as the point of origin for several muscles that move the arms, head, and neck. Chest Wall: Anatomy, and clotting deficiencies
    • Treacher Collins syndrome Treacher Collins Syndrome Treacher Collins syndrome is a rare genetic condition with autosomal dominant inheritance. Treacher Collins syndrome is also referred to as mandibulofacial dysostosis or Franceschetti syndrome and is characterized by significant craniofacial deformities and conductive hearing loss. Treacher Collins Syndrome: autosomal dominant Autosomal dominant Autosomal inheritance, both dominant and recessive, refers to the transmission of genes from the 22 autosomal chromosomes. Autosomal dominant diseases are expressed when only 1 copy of the dominant allele is inherited. Autosomal Recessive and Autosomal Dominant Inheritance, associated with craniofacial deformities and conductive hearing loss Conductive hearing loss Hearing loss due to interference with the mechanical reception or amplification of sound to the cochlea. The interference is in the outer or middle ear involving the ear canal; tympanic membrane; or ear ossicles. Hearing Loss
  • Ostium primum is mainly seen in patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship with Down’s syndrome.

Related videos

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Atrial Septal Defects

Pathophysiology

  • Because the pressure in the LA is higher, the blood flows from the LA to the RA through the patent interatrial septum at the end of late systole Systole Period of contraction of the heart, especially of the heart ventricles. Cardiac Cycle and early diastole Diastole Post-systolic relaxation of the heart, especially the heart ventricles. Cardiac Cycle, forming a left-to-right shunt (L–R).
  • The L–R shunt leads to an increase in volume in the right side of the heart, resulting in:
    • Pulmonary overcirculation → pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressure, which can lead to chronic progressive right heart failure. Pulmonary hypertension is grouped into 5 categories based on etiology, which include primary PAH, and PH due to cardiac disease, lung or hypoxic disease, chronic thromboembolic disease, and multifactorial or unclear etiologies. Pulmonary Hypertension → hypertrophy Hypertrophy General increase in bulk of a part or organ due to cell enlargement and accumulation of fluids and secretions, not due to tumor formation, nor to an increase in the number of cells (hyperplasia). Cellular Adaptation of the right ventricle (RV)
    • Dilatation of the RA → supraventricular arrhythmias (e.g., atrial flutter Atrial flutter Atrial flutter is a regular supraventricular tachycardia characterized by an atrial heart rate between 240/min and 340/min (typically 300/min), atrioventricular (AV) node conduction block, and a “sawtooth” pattern on an electrocardiogram (ECG). Atrial Flutter and fibrillation) 
  • Eventually, the RV hypertrophy Hypertrophy General increase in bulk of a part or organ due to cell enlargement and accumulation of fluids and secretions, not due to tumor formation, nor to an increase in the number of cells (hyperplasia). Cellular Adaptation causes an increase in pressure in the right side → the shunt is reversed → Eisenmenger’s syndrome occurs
    • Eisenmenger’s syndrome: a condition in which an initially acyanotic Acyanotic Tetralogy of Fallot heart defect with an L–R shunt is reversed by the right-sided hypertrophy Hypertrophy General increase in bulk of a part or organ due to cell enlargement and accumulation of fluids and secretions, not due to tumor formation, nor to an increase in the number of cells (hyperplasia). Cellular Adaptation; an R–L shunt is created, leading to cyanosis Cyanosis A bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule. Pulmonary Examination
  • The L–R shunt also results in decreased blood volume being pumped into the systemic circulation Circulation The movement of the blood as it is pumped through the cardiovascular system. ABCDE Assessment → heart failure Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (ventricular dysfunction), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as myocardial infarction. Total Anomalous Pulmonary Venous Return (TAPVR)
  • The larger the diameter of the defect, the bigger the blood flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure and the more severe the symptoms will be.
Pathophysiology of atrial septal defect (asd)

Pathophysiology of atrial septal defect
The image depicts the change in blood flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure in the presence of an atrial septal defect ( ASD ASD Autism spectrum disorder (ASD) is a neurodevelopmental disorder marked by poor social skills, restricted interests/social interactions, and repetitive/stereotyped behaviors. The condition is termed a “spectrum” because of the wide variability in the severity of symptoms exhibited. Autism Spectrum Disorder). Note the decreased blood flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure to the left ventricle (7) and the increased blood flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure into the pulmonary circulation Circulation The movement of the blood as it is pumped through the cardiovascular system. ABCDE Assessment (11).

Image by Lecturio.

Clinical Presentation

Signs and symptoms

The signs and symptoms depend on the size of the defect and the severity of the shunt:

  • Small defects are usually asymptomatic.
  • Infants and children with smaller defects may be diagnosed incidentally:
  • Larger defects present in infancy or young children with:
    • Failure to thrive Failure to Thrive Failure to thrive (FTT), or faltering growth, describes suboptimal weight gain and growth in children. The majority of cases are due to inadequate caloric intake; however, genetic, infectious, and oncological etiologies are also common. Failure to Thrive
    • Recurrent lung infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease
    • Symptoms of heart failure Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (ventricular dysfunction), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as myocardial infarction. Total Anomalous Pulmonary Venous Return (TAPVR):
      • Dyspnea Dyspnea Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea during physical exertion
      • Tiring/ fatigue Fatigue The state of weariness following a period of exertion, mental or physical, characterized by a decreased capacity for work and reduced efficiency to respond to stimuli. Fibromyalgia when feeding (mostly in infants)
      • Difficulty breathing when lying down
      • Swelling Swelling Inflammation of the legs
    • Paradoxical embolism can occur with ASDs: An embolus can enter the systemic circulation Circulation The movement of the blood as it is pumped through the cardiovascular system. ABCDE Assessment through the shunt, resulting in a stroke or acute limb ischemia Acute limb ischemia Acute limb ischemia (ALI) is a major vascular emergency because of the rapid decrease in limb perfusion that causes a potential threat to limb viability. The majority of cases are caused by arterial thrombosis due to plaque progression or embolism, but ALI can also be caused by blockage of the venous drainage. Acute Limb Ischemia.

Findings upon physical examination

Upon physical examination, the following can be seen/detected:

  • General appearance: The patient may have dyspnea Dyspnea Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea or lethargy Lethargy A general state of sluggishness, listless, or uninterested, with being tired, and having difficulty concentrating and doing simple tasks. It may be related to depression or drug addiction. Hyponatremia.
  • Precordial bulge due to atrial enlargement
  • Murmur:
    • Increased flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure at the pulmonary valve Pulmonary valve A valve situated at the entrance to the pulmonary trunk from the right ventricle. Heart: Anatomy → mid-systolic ejection murmur (over the 2nd intercostal space)
    • Increased flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure at the tricuspid valve Tricuspid valve The valve consisting of three cusps situated between the right atrium and right ventricle of the heart. Heart: Anatomy → soft mid-diastolic murmur (over the lower left sternum Sternum A long, narrow, and flat bone commonly known as breastbone occurring in the midsection of the anterior thoracic segment or chest region, which stabilizes the rib cage and serves as the point of origin for several muscles that move the arms, head, and neck. Chest Wall: Anatomy)
    • Associated mitral regurgitation Regurgitation Gastroesophageal Reflux Disease (GERD) → holosystolic murmur Holosystolic Murmur Tricuspid Valve Atresia (TVA) (over the apex)
  • Fixed, wide split S2 S2 Heart Sounds
  • Signs of pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressure, which can lead to chronic progressive right heart failure. Pulmonary hypertension is grouped into 5 categories based on etiology, which include primary PAH, and PH due to cardiac disease, lung or hypoxic disease, chronic thromboembolic disease, and multifactorial or unclear etiologies. Pulmonary Hypertension:
  • Signs and symptoms of heart failure Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (ventricular dysfunction), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as myocardial infarction. Total Anomalous Pulmonary Venous Return (TAPVR):
    • S3 S3 Heart Sounds and S4 S4 Heart Sounds heart sounds Heart sounds Heart sounds are brief, transient sounds produced by valve opening and closure and by movement of blood in the heart. They are divided into systolic and diastolic sounds. In most cases, only the first (S1) and second (S2) heart sounds are heard. These are high-frequency sounds and arise from aortic and pulmonary valve closure (S1), as well as mitral and tricuspid valve closure (S2). Heart Sounds
    • Displaced cardiac Cardiac Total Anomalous Pulmonary Venous Return (TAPVR) apex
    • Edematous lower limb
    • Hepatic congestion → ascites Ascites Ascites is the pathologic accumulation of fluid within the peritoneal cavity that occurs due to an osmotic and/or hydrostatic pressure imbalance secondary to portal hypertension (cirrhosis, heart failure) or non-portal hypertension (hypoalbuminemia, malignancy, infection). Ascites
    • Hepatomegaly
Cardiac murmurs after correction

Phonocardiograms of abnormal heart sounds Heart sounds Heart sounds are brief, transient sounds produced by valve opening and closure and by movement of blood in the heart. They are divided into systolic and diastolic sounds. In most cases, only the first (S1) and second (S2) heart sounds are heard. These are high-frequency sounds and arise from aortic and pulmonary valve closure (S1), as well as mitral and tricuspid valve closure (S2). Heart Sounds caused by the following cardiac Cardiac Total Anomalous Pulmonary Venous Return (TAPVR) defects:
aortic regurgitation Regurgitation Gastroesophageal Reflux Disease (GERD), mitral valve prolapse Mitral valve prolapse Abnormal protrusion or billowing of one or both of the leaflets of mitral valve into the left atrium during systole. This allows the backflow of blood into left atrium leading to mitral valve insufficiency, systolic murmurs, or cardiac arrhythmia. Mitral Valve Prolapse, mitral stenosis Stenosis Hypoplastic Left Heart Syndrome (HLHS) ( MS MS Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis), aortic stenosis Stenosis Hypoplastic Left Heart Syndrome (HLHS) (AS), tricuspid regurgitation Regurgitation Gastroesophageal Reflux Disease (GERD), hypertrophic obstructive cardiomyopathy Cardiomyopathy Cardiomyopathy refers to a group of myocardial diseases associated with structural changes of the heart muscles (myocardium) and impaired systolic and/or diastolic function in the absence of other heart disorders (coronary artery disease, hypertension, valvular disease, and congenital heart disease). Cardiomyopathy: Overview and Types (HOCM), atrial septal defect ( ASD ASD Autism spectrum disorder (ASD) is a neurodevelopmental disorder marked by poor social skills, restricted interests/social interactions, and repetitive/stereotyped behaviors. The condition is termed a “spectrum” because of the wide variability in the severity of symptoms exhibited. Autism Spectrum Disorder), ventricular septal defect Ventricular Septal Defect Tetralogy of Fallot (VSD), and patent ductus arteriosus Patent ductus arteriosus The ductus arteriosus (DA) allows blood to bypass pulmonary circulation. After birth, the DA remains open for up to 72 hours and then constricts and involutes, becoming the ligamentum arteriosum. Failure of this process to occur results in patent ductus arteriosus (PDA), a condition that causes up to 10% of congenital heart defects. Patent Ductus Arteriosus (PDA) ( PDA PDA The ductus arteriosus (DA) allows blood to bypass pulmonary circulation. After birth, the DA remains open for up to 72 hours and then constricts and involutes, becoming the ligamentum arteriosum. Failure of this process to occur results in patent ductus arteriosus (PDA), a condition that causes up to 10% of congenital heart defects. Patent Ductus Arteriosus (PDA))

Image by Lecturio.

Audio:

This audio file is an example of an ejection murmur and split S2 S2 Heart Sounds heard over the 2nd left intercostal space in an individual with an atrial septal defect. The crescendo-decrescendo murmur is heard between S1 S1 Heart Sounds and S2 S2 Heart Sounds, the latter of which is widely split.

Heart sound by The Regents of the University of Michigan. License: CC BY-SA 3.0

Diagnosis and Management

Diagnosis

  • Echocardiogram Echocardiogram Transposition of the Great Vessels confirms the diagnosis by detecting:
    • Size of the defect
    • Volume
    • Shunt ratio
    • Pulmonary arterial pressures
  • Other supportive tests include:
    • Electrocardiogram Electrocardiogram An electrocardiogram (ECG) is a graphic representation of the electrical activity of the heart plotted against time. Adhesive electrodes are affixed to the skin surface allowing measurement of cardiac impulses from many angles. The ECG provides 3-dimensional information about the conduction system of the heart, the myocardium, and other cardiac structures. Electrocardiogram (ECG) ( ECG ECG An electrocardiogram (ECG) is a graphic representation of the electrical activity of the heart plotted against time. Adhesive electrodes are affixed to the skin surface allowing measurement of cardiac impulses from many angles. The ECG provides 3-dimensional information about the conduction system of the heart, the myocardium, and other cardiac structures. Electrocardiogram (ECG)) (changes are only seen in a large defect):
      • Incomplete right bundle branch block Right bundle branch block Bundle Branch and Fascicular Blocks
      • Supraventricular tachycardia Tachycardia Abnormally rapid heartbeat, usually with a heart rate above 100 beats per minute for adults. Tachycardia accompanied by disturbance in the cardiac depolarization (cardiac arrhythmia) is called tachyarrhythmia. Sepsis in Children ( atrial fibrillation Atrial fibrillation Atrial fibrillation (AF or Afib) is a supraventricular tachyarrhythmia and the most common kind of arrhythmia. It is caused by rapid, uncontrolled atrial contractions and uncoordinated ventricular responses. Atrial Fibrillation or  atrial flutter Atrial flutter Atrial flutter is a regular supraventricular tachycardia characterized by an atrial heart rate between 240/min and 340/min (typically 300/min), atrioventricular (AV) node conduction block, and a “sawtooth” pattern on an electrocardiogram (ECG). Atrial Flutter)
    • Chest X-ray X-ray Penetrating electromagnetic radiation emitted when the inner orbital electrons of an atom are excited and release radiant energy. X-ray wavelengths range from 1 pm to 10 nm. Hard x-rays are the higher energy, shorter wavelength x-rays. Soft x-rays or grenz rays are less energetic and longer in wavelength. The short wavelength end of the x-ray spectrum overlaps the gamma rays wavelength range. The distinction between gamma rays and x-rays is based on their radiation source. Pulmonary Function Tests:
      • Prominent pulmonary vasculature
      • Cardiomegaly Cardiomegaly Enlargement of the heart, usually indicated by a cardiothoracic ratio above 0. 50. Heart enlargement may involve the right, the left, or both heart ventricles or heart atria. Cardiomegaly is a nonspecific symptom seen in patients with chronic systolic heart failure (heart failure) or several forms of cardiomyopathies. Ebstein’s Anomaly
  • Ultrasound of woman with atrial septal defect

    Transthoracic echocardiography Echocardiography Ultrasonic recording of the size, motion, and composition of the heart and surrounding tissues. The standard approach is transthoracic. Tricuspid Valve Atresia (TVA) of ASD ASD Autism spectrum disorder (ASD) is a neurodevelopmental disorder marked by poor social skills, restricted interests/social interactions, and repetitive/stereotyped behaviors. The condition is termed a “spectrum” because of the wide variability in the severity of symptoms exhibited. Autism Spectrum Disorder, in apical 4-chamber view:
    AD AD The term advance directive (AD) refers to treatment preferences and/or the designation of a surrogate decision-maker in the event that a person becomes unable to make medical decisions on their own behalf. Advance directives represent the ethical principle of autonomy and may take the form of a living will, health care proxy, durable power of attorney for health care, and/or a physician’s order for life-sustaining treatment. Advance Directives: right atrium
    AS: left atrium
    VD: right ventricle
    A0: aorta Aorta The main trunk of the systemic arteries. Mediastinum and Great Vessels: Anatomy
    DSA: atrial septal defect

    Image: “2D Transthoracic echocardiography Echocardiography Ultrasonic recording of the size, motion, and composition of the heart and surrounding tissues. The standard approach is transthoracic. Tricuspid Valve Atresia (TVA)” by the Carol Davila University of Medicine and Pharmacy, Bucharest, Romania. License: CC BY 2.0.
  • Sinus venosus atrial septal defect complicated by eisenmenger syndrome

    A case of sinus venosus ASD ASD Autism spectrum disorder (ASD) is a neurodevelopmental disorder marked by poor social skills, restricted interests/social interactions, and repetitive/stereotyped behaviors. The condition is termed a “spectrum” because of the wide variability in the severity of symptoms exhibited. Autism Spectrum Disorder complicated by Eisenmenger’s syndrome:
    A chest radiograph shows a markedly enlarged pulmonary trunk Pulmonary Trunk Truncus Arteriosus (red arrow), enlarged central pulmonary arteries Arteries Arteries are tubular collections of cells that transport oxygenated blood and nutrients from the heart to the tissues of the body. The blood passes through the arteries in order of decreasing luminal diameter, starting in the largest artery (the aorta) and ending in the small arterioles. Arteries are classified into 3 types: large elastic arteries, medium muscular arteries, and small arteries and arterioles. Arteries: Histology, and enlargement of the cardiac Cardiac Total Anomalous Pulmonary Venous Return (TAPVR) silhouette.

    Image: “Chest radiograph” by Amornpol Anuwatworn et al AL Amyloidosis. License: CC BY 4.0.

Management

  • Yearly follow-up for cases of ostium secundum sub-type < 3 mm MM Multiple myeloma (MM) is a malignant condition of plasma cells (activated B lymphocytes) primarily seen in the elderly. Monoclonal proliferation of plasma cells results in cytokine-driven osteoclastic activity and excessive secretion of IgG antibodies. Multiple Myeloma that close spontaneously by 2 years of age (most cases)
  • Medical management for symptomatic ASDs:
    • Nutritional support
    • Manage heart failure Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (ventricular dysfunction), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as myocardial infarction. Total Anomalous Pulmonary Venous Return (TAPVR).
    • Manage pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressure, which can lead to chronic progressive right heart failure. Pulmonary hypertension is grouped into 5 categories based on etiology, which include primary PAH, and PH due to cardiac disease, lung or hypoxic disease, chronic thromboembolic disease, and multifactorial or unclear etiologies. Pulmonary Hypertension.
    • Antibiotic prophylaxis Prophylaxis Cephalosporins until 6 months post-surgical repair 
  • Surgical patching percutaneous repair: if the patient is symptomatic despite medical management
  • Lung and heart transplant Heart transplant The transference of a heart from one human or animal to another. Dilated Cardiomyopathy: if Eisenmenger’s syndrome has occurred

Prognosis Prognosis A prediction of the probable outcome of a disease based on a individual’s condition and the usual course of the disease as seen in similar situations. Non-Hodgkin Lymphomas

  • Percutaneous closure has a success rate of 88%–98%. 
  • Participation in sports depends on the state of the ASD ASD Autism spectrum disorder (ASD) is a neurodevelopmental disorder marked by poor social skills, restricted interests/social interactions, and repetitive/stereotyped behaviors. The condition is termed a “spectrum” because of the wide variability in the severity of symptoms exhibited. Autism Spectrum Disorder and if there is pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressure, which can lead to chronic progressive right heart failure. Pulmonary hypertension is grouped into 5 categories based on etiology, which include primary PAH, and PH due to cardiac disease, lung or hypoxic disease, chronic thromboembolic disease, and multifactorial or unclear etiologies. Pulmonary Hypertension.

Clinical Relevance

The following conditions are associated with ASDs and can cause or modify the clinical features of ASDs: 

  • Failure to thrive Failure to Thrive Failure to thrive (FTT), or faltering growth, describes suboptimal weight gain and growth in children. The majority of cases are due to inadequate caloric intake; however, genetic, infectious, and oncological etiologies are also common. Failure to Thrive: suboptimal weight gain Suboptimal Weight Gain Failure to Thrive and growth in children. As with the majority of CHDs, the 1st presentation Presentation The position or orientation of the fetus at near term or during obstetric labor, determined by its relation to the spine of the mother and the birth canal. The normal position is a vertical, cephalic presentation with the fetal vertex flexed on the neck. Normal and Abnormal Labor of ASD ASD Autism spectrum disorder (ASD) is a neurodevelopmental disorder marked by poor social skills, restricted interests/social interactions, and repetitive/stereotyped behaviors. The condition is termed a “spectrum” because of the wide variability in the severity of symptoms exhibited. Autism Spectrum Disorder may be of an infant or child who is failing to meet the necessary caloric requirement for growth due to the increased demand of a failing heart and decreased blood flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure as seen in ASDs.
  • Pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressure, which can lead to chronic progressive right heart failure. Pulmonary hypertension is grouped into 5 categories based on etiology, which include primary PAH, and PH due to cardiac disease, lung or hypoxic disease, chronic thromboembolic disease, and multifactorial or unclear etiologies. Pulmonary Hypertension: characterized by elevated pulmonary arterial pressure Elevated pulmonary arterial pressure. Pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressure, which can lead to chronic progressive right heart failure. Pulmonary hypertension is grouped into 5 categories based on etiology, which include primary pah, and ph due to cardiac disease, lung or hypoxic disease, chronic thromboembolic disease, and multifactorial or unclear etiologies. Pulmonary Hypertensiondue to pulmonary overcirculation. Pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressure, which can lead to chronic progressive right heart failure. Pulmonary hypertension is grouped into 5 categories based on etiology, which include primary PAH, and PH due to cardiac disease, lung or hypoxic disease, chronic thromboembolic disease, and multifactorial or unclear etiologies. Pulmonary Hypertension is a common complication of large ASDs. Hypertension Hypertension Hypertension, or high blood pressure, is a common disease that manifests as elevated systemic arterial pressures. Hypertension is most often asymptomatic and is found incidentally as part of a routine physical examination or during triage for an unrelated medical encounter. Hypertension causes the majority of the symptoms of ASD ASD Autism spectrum disorder (ASD) is a neurodevelopmental disorder marked by poor social skills, restricted interests/social interactions, and repetitive/stereotyped behaviors. The condition is termed a “spectrum” because of the wide variability in the severity of symptoms exhibited. Autism Spectrum Disorder and is the main pathology behind Eisenmenger’s syndrome.
  • Heart failure Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (ventricular dysfunction), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as myocardial infarction. Total Anomalous Pulmonary Venous Return (TAPVR): the inability of the heart to supply the body with normal cardiac Cardiac Total Anomalous Pulmonary Venous Return (TAPVR) output to meet metabolic needs. The decreased volume into the left ventricle and, therefore, reduced stroke volume Stroke volume The amount of blood pumped out of the heart per beat, not to be confused with cardiac output (volume/time). It is calculated as the difference between the end-diastolic volume and the end-systolic volume. Cardiac Cycle result in ventricular remodeling to maintain cardiac Cardiac Total Anomalous Pulmonary Venous Return (TAPVR) output. With time, the cardiac Cardiac Total Anomalous Pulmonary Venous Return (TAPVR) pump Pump ACES and RUSH: Resuscitation Ultrasound Protocols fails to keep up, and patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship may present with heart failure Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (ventricular dysfunction), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as myocardial infarction. Total Anomalous Pulmonary Venous Return (TAPVR) in adulthood.
  • Ebstein’s anomaly: a cyanotic CHD characterized by the downward displacement Displacement The process by which an emotional or behavioral response that is appropriate for one situation appears in another situation for which it is inappropriate. Defense Mechanisms of the septal and posterior leaflets of the tricuspid valve Tricuspid valve The valve consisting of three cusps situated between the right atrium and right ventricle of the heart. Heart: Anatomy. One of the associated features of Ebstein’s anomaly is an interatrial defect. In cases where an ASD ASD Autism spectrum disorder (ASD) is a neurodevelopmental disorder marked by poor social skills, restricted interests/social interactions, and repetitive/stereotyped behaviors. The condition is termed a “spectrum” because of the wide variability in the severity of symptoms exhibited. Autism Spectrum Disorder presents with cyanosis Cyanosis A bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule. Pulmonary Examination, an echocardiogram Echocardiogram Transposition of the Great Vessels must be done to rule out Ebstein’s anomaly.

References

  1. Kliegman, R. M. et al. (2020). Nelson textbook of pediatrics (pp. 237–2384.e1). http://dx.doi.org/10.1016/B978-0-323-52950-1.00453-3
  2. Silvestry, F.E. et al. (2015). Guidelines for the echocardiographic assessment of atrial septal defect and patent foramen ovale: From the American society of echocardiography and society for cardiac angiography and interventions. Journal of the American Society of Echocardiography, 28(8), 910-958. http://dx.doi.org/10.1016/j.echo.2015.05.015
  3. Jain, S., & Dalvi, B. (2018). Atrial septal defect with pulmonary hypertension: When/how can we consider closure? Journal of thoracic disease, 10(Suppl 24), S2890–S2898. hhttp://dx.doi.org/10.21037/jtd.2018.07.112

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