Acyanotic congenital heart defects

Acyanotic congenital heart defects (CHDs) are cardiac malformations that affect the atrial or ventricular walls, heart valves, or large blood vessels. Common causes include genetic defects (e.g., trisomies), maternal infections (e.g., rubella), or maternal use of drugs or alcohol during pregnancy. Acyanotic heart defects are pathophysiologically characterized by a left-to-right shunt, which causes pulmonary hypertension and right heart hypertrophy. The symptoms depend on the extent of the malformation and the resulting impairment of cardiac function. Infants may be asymptomatic or present with exercise intolerance, failure to thrive, and symptoms of heart failure. Characteristic heart murmurs are important clues for establishing the diagnosis, which is typically confirmed by visualizing the defect on echocardiography. Chest x-ray, MRI, or cardiac catheterization may also be required to determine indications for surgery and plan the procedure. Acyanotic heart defects requiring treatment are repaired via catheter procedures or surgery. Supportive medical therapy is required in cases of heart failure (e.g., diuretics, inotropic agents) or if surgery cannot be performed (e.g., prostaglandin). Complications are more likely the longer treatment is delayed, and include arrhythmias, embolisms, infective endocarditis, and reversal of shunt physiology (i.e., Eisenmenger syndrome).

Common acyanotic CHDs

The “3 Ds” of acyanotic CHDs (in order of frequency): VSD, ASD, PDA

Pathogenesis ^[19]

Principles

• Congenital heart defects (CHDs) are caused by the disruption of the normal sequence of cardiac morphogenesis.
• CHDs may lead to the formation of pathological connections (shunts) between the right and left heart chambers, allowing blood to flow along the pressure gradient from high pressure to low pressure.
• The shunts are classified according to the direction of the blood flow as either left-to-right or right-to-left.

Shunt types

• Left-to-right shunt
  • Oxygenated blood from the lungs is shunted back into the pulmonary circulation via an atrial septal defect (ASD), ventricular septal defect (VSD), or patent ductus arteriosus (PDA) → pulmonary hypertension
  • Right ventricular pressure overload → right-sided heart hypertrophy (cardiomegaly on x-ray) and heart failure but no cyanosis
• Right-to-left shunt: blood flows from the right to the left heart via a shunt → deoxygenated blood entering the systemic circulation → cyanosis
• See “Eisenmenger syndrome” for details on how a left-to-right shunt can develop into a right-to-left shunt over time.

Left-to-Right shunts = LateR cyanosis. Right-to-Left shunts = eaRLy cyanosis.

General clinical features

For specific features, see “Clinical features” in the respective subsections.

Nonspecific findings

• Failure to thrive
• Recurrent bronchopulmonary infections
• Normal skin tone
• Exercise intolerance
  • Fatigue, pallor, and diaphoresis (sweating)
  • Tachycardia
  • Dyspnea
  • Grunting, nasal flaring, retractions, and/or head bobbing may be seen ^[20]

Heart failure

• Right heart failure
  • Hepatic venous congestion with hepatomegaly
  • Peripheral edema is rarely seen in infants. ^[21]
• Left heart failure
  • Tachypnea, pulmonary edema
  • Low cardiac output: ↓ blood pressure, pallor, sweating, cool extremities, syncope

Medical management of acyanotic CHDs ^[3]

• Nutritional support and routine vaccinations
• Ductal-dependent CHDs: prostaglandin E1 infusion to prevent PDA closure
• Manage heart failure.
  • Respiratory support as needed
  • Diuretics to decrease fluid volume
  • ACE inhibitors to lower systemic vascular resistance
  • Inotropic agents (e.g., digoxin) to improve cardiac contractility
• Determine the need for surgical repair and postoperative antibiotic prophylaxis.
• Manage Eisenmenger syndrome if it develops.

Description

• A defect in the atrial wall that may result from impaired growth or excessive resorption of the atrial septum

Epidemiology ^[3]

• Third most common CHD (∼ 1/2,000 live births) ^[22]
• Sex: ♀ > ♂

Etiology

• Down syndrome ^[4]
• Fetal alcohol syndrome ^[5]
• Intrauterine infections (e.g., TORCH) ^[6][7]
• Holt-Oram syndrome (hand-heart syndrome) ^[3]
  • Autosomal dominant disorder
  • Affects ∼ 1/100,000 children
  • Characterized by ASD, a first-degree heart block, and abnormalities of the upper limbs (e.g., absent radial bones)

Pathophysiology

• Impaired growth or excessive resorption of the atrial septa in utero leads to atrial septal defects (absent atrial septa tissue).
  • Ostium primum atrial septal defect (ASD I): ∼ 15–20% (usually accompanied by other heart defects)
  • Ostium secundum atrial septal defect (ASD II): ∼ 70% (usually isolated)
• Typically a low-pressure, low-volume, minor left-to-right shunt (therefore, patients are asymptomatic)
• ASD → oxygenated blood shunting from LA to RA → ↑ O₂ saturation in the RA → ↑ O₂ saturation in RV and pulmonary artery
• In more severe defects, the shunts may lead to supraventricular arrhythmias, pulmonary hypertension, and/or Eisenmenger syndrome.

Clinical features ^[3]

General

• Depend on defect size and shunt volume
• Small defects: usually asymptomatic
• Medium-sized to large defects
  • Symptoms can vary from asymptomatic to heart failure.
  • ASDs typically manifest with advancing age. ^[3]
• See “General clinical features” above.

Auscultation ^[3]

• Systolic ejection murmur over the second left ICS sternal border
• Widely split second heart sound (S₂) over the second left ICS,; which is fixed (does not change with respiration)
• Soft mid-diastolic murmur over the lower left sternal border

Diagnostics ^[23]

• Echocardiography (confirmatory test): interatrial communication, best visualized in the apical four-chamber and subcostal views ; : ^[24]
• ECG: signs of RV hypertrophy (vertical or right axis deviation, P pulmonale, PR prolongation, complete or incomplete right bundle branch block)
• Chest x-ray
  • Enlarged right atrium, right ventricle, and pulmonary artery
  • Enhanced pulmonary vasculature

Treatment ^[3]

• Medical management: rarely required
• Surgical repair (e.g., transcatheter closure, patch) for:
  • Right ventricular enlargement
  • Large left-to-right shunts
  • Clinical features of congestive heart failure
  • Prior paradoxical embolism

Up to 40% of ASDs spontaneously close by the age of 5 years. ^[25]

Complications ^[26]

• Paradoxical embolism (↑ risk of ischemic stroke): small blood portions from inferior vena cava bypass pulmonary circulation → direct emptying into left atrium → paradoxical embolism and stroke in the case of thromboembolism
• Heart failure

Description

• An abnormal communication between the left and right ventricle that results in left-to-right shunting of blood flow

Epidemiology

• Most common congenital heart defect (∼ 4/1,000 live births) ^[27]
• Occurs as an isolated heart defect or in combination with others (e.g., with AVSD, tetralogy of Fallot, TGA)

Etiology

• Genetic syndromes ^[3]
  • Most commonly: Down syndrome, Edward syndrome, Patau syndrome
  • Less commonly: Cri-du-chat syndrome, Apert syndrome
• Intrauterine infections (e.g., TORCH) ^[6][7]
• Maternal risk factors: diabetes, obesity, smoking ^[9][27]

Pathophysiology

• Most commonly located in the membranous part of the ventricular septum (pars membranacea)
• Defect in ventricular septum → left-to-right shunt with the following consequences:
  • RV volume overload → RV eccentric hypertrophy
  • Excessive pulmonary blood flow → ↑ pulmonary artery pressure → pulmonary hypertension
  • ↓ Cardiac output
  • LV volume overload → LV eccentric hypertrophy
  • ↑ O₂ saturation in right ventricle and pulmonary artery

Clinical features ^[3][8]

General

• Small defects: usually asymptomatic
• Medium-sized or large defects
  • Lead to heart failure by the age of 2–3 months
  • Become symptomatic after high pulmonary vascular resistance (PVR) present at birth starts to decrease: ↓ PVR → ↓ right ventricular pressure → ↑ left-to-right shunt → symptoms
  • See “Nonspecific findings” and “Heart failure” in “Overview” above.
• Hyperdynamic precordium may be detected in hemodynamically relevant defects.

Auscultation

• Harsh holosystolic murmur over the left lower sternal border
  • Becomes more intense with maneuvers that increase left ventricular afterload (e.g., handgrip)
  • Typically louder in small defects
• Mid-diastolic murmur over cardiac apex
• Loud pulmonic S₂ (if pulmonary hypertension develops)
• Systolic thrill

Symptoms of heart failure in children with VSD only develop when PVR decreases to adult levels and thus allows left-to-right shunting to occur.

Diagnostics ^[8]

• Echocardiography (confirmatory test)
  • To assess defect size and shunt volume
  • Doppler ultrasound findings of pathological left-to-right blood flow can help diagnose minor ventricular defects.
• ECG
  • Small defects: normal ECG findings
  • Medium-sized or large defects
    • Signs of LV hypertrophy (due to volume loading): ↑ QRS amplitude, left axis deviation, left atrial enlargement
    • Signs of RV hypertrophy (due to pulmonary hypertension or obstruction of the pulmonary outflow tract): vertical or right axis deviation, P pulmonale, PR prolongation, complete or incomplete right bundle branch block
• Chest x-ray
  • Enhanced pulmonary vascular markings
  • Left atrial and ventricular enlargement
  • In later stages, enlarged right ventricle and pulmonary artery (due to elevated PVR)

Treatment ^[3][8]

• Small defects
  • Often close spontaneously and rarely require surgical interventions
  • Follow-up echocardiography is recommended.
• Symptomatic and large defects
  • All patients: Provide medical management of acyanotic CHDs as needed.
  • Surgical repair (e.g., patch)
    • Infants with large left-to-right shunts with clinical symptoms (e.g., failure to thrive, treatment-resistant congestive heart failure)
    • Asymptomatic older children with large left-to-right shunts or evidence of left atrium or left ventricular dilatation
    • VSD with aortic insufficiency or aortic valve prolapse into the VSD
    • Pulmonary hypertension

VSD closure results in lower right ventricular and left atrial pressures and higher left ventricular pressures than preclosure values. ^[28]

VSD closure is contraindicated in patients with Eisenmenger syndrome. ^[3]

Complications

• Arrhythmias
• Heart failure
• Eisenmenger syndrome
• Infective endocarditis
• Aortic regurgitation

Definition ^[3]

A defect of atrioventricular valves (i.e., mitral and tricuspid valves) as well as the atrial and/or ventricular septum; previously referred to as endocardial cushion defects

• Complete form: ASD and VSD, common AV valve
• Partial form: only ASD and minor AV valve abnormalities

Etiology

• Strongly associated with Down syndrome ^[29][30]
• Association with maternal diabetes and obesity has been shown in some studies. ^[31]

Pathophysiology ^[3]

• Complete form: (ASD and VSD) → atrial and ventricular left-to-right shunt → excessive pulmonary blood flow and biventricular volume overload → pulmonary hypertension and heart failure
• Partial form (ASD only) → atrial left-to-right shunt → symptoms that may remain minimal until adulthood
• In both forms: abnormal AV valve → AV valve regurgitation → in utero heart failure (nonimmune hydrops fetalis) ^[32]

Clinical features ^[3]

• Complete form: See “Nonspecific findings” and “Heart failure” in “Overview” above.
• Partial form: See “Clinical features” in “Atrial septal defect (ASD).“

Diagnostics ^[33]

• Antenatal echocardiography: findings of endocardial cushion defect in first trimester → screening for Down syndrome
• Echocardiography (confirmatory test): to assess defect size, shunt volume, and global cardiac function
• ECG: left axis deviation due to LVH
• Chest x-ray
  • Complete form: global cardiomegaly, ↑ pulmonary markings
  • Partial form: enlarged right heart and pulmonary artery

Treatment ^[3][14][33]

• All patients: Provide medical management of acyanotic CHDs as needed.
• Surgical management: patch closure and AV valve reconstruction; generally indicated unless Eisenmenger syndrome has developed
  • Complete form: generally between 3–6 months of age
  • Partial form: generally between 2–4 years of age
  • Older patients: elective procedure

Description

• A variant of cardiac anatomy in which the foramen ovale remains patent beyond 1 year of age

Epidemiology

• Prevalence: ∼25% of the general population ^[34]

Etiology

• Associated with Loeys-Dietz syndrome

Pathophysiology

• Failure of the atrial septum primum to fuse with the septum secundum following birth → persistence of foramen ovale; → mild left-to-right shunt
• A shunt reversal may be induced by certain maneuvers that increase right atrial pressure (e.g., Valsalva maneuver, coughing).

ASD = Septal tissue Deficiency. PFO = enough tissue, but Problems with Fusion.

Clinical features

• Affected individuals are usually asymptomatic until complications occur.

Diagnostics ^[35]

PFOs are sometimes an incidental finding during procedures (e.g., PCI, cardiac surgery) or must be evaluated for after an embolic event (e.g., ischemic stroke).

• TTE with agitated saline (best initial test): to evaluate for a right-to-left shunt
• Indications for TEE with agitated saline
  • Inconclusive TTE
  • High index of suspicion for PFO despite negative TTE
  • Need for PFO anatomic assessment after positive TTE

Treatment ^[10]

• Asymptomatic PFO: Treatment is not usually required.
• Confirmed PFO after embolic event (e.g., ischemic stroke) ^[10]
  • Requires multidisciplinary evaluation by cardiology and neurology to determine the likelihood of paradoxical embolism from PFO
  • Treatment may include:
    • Antiplatelet agents or anticoagulation
    • Surgical or percutaneous closure of the defect

PFO is one of many possible causes of stroke. Reducing subsequent stroke risk should include the evaluation of other potential causes (e.g., arrhythmia, hypercoagulability, endocarditis). ^[10]

Complications ^[36]

• Paradoxical embolism
• Ischemic stroke
• Systemic embolisms (e.g., renal infarction)

Description

• Failure of the ductus arteriosus to completely close postnatally

Epidemiology

• Incidence: 5–10% full-term births ^[37]
• In premature infants: 20–60% ^[37]
• Sex: ♀ > ♂ (2:1) ^[3]

Etiology

• Prematurity
• Maternal exposure during pregnancy
  • Rubella infection (during the first trimester of pregnancy)
  • Alcohol consumption
  • Phenytoin use (fetal hydantoin syndrome)
  • Prostaglandin use
• Respiratory distress syndrome
• Trisomies (e.g., Down syndrome)

Pathophysiology

• Ductus arteriosus enables the underdeveloped lungs to be bypassed by the fetal circulation (normal right-to-left shunt) and remains patent in utero via PGE and low O₂ tension.
• After birth, pulmonary vascular resistance decreases and thus allows for the reversal of the shunt from right-to-left to left-to-right.
• Failure of the ductus arteriosus to close after birth → persistent communication between the aorta and the pulmonary artery → left-to-right shunt → volume overload of the pulmonary vessels → continuous RV (and/or LV) strain → heart failure (see also “Overview” above)
• Eisenmenger syndrome may occur with shunt reversal and manifest with differential cyanosis.

Clinical features

General

• Small PDA: asymptomatic with normal findings on physical examination
• Large PDA
  • Nonspecific symptoms (e.g., failure to thrive) and symptoms of heart failure in infancy (see the “Overview” above)
  • Bounding peripheral pulses, wide pulse pressure ^[38]
  • Heaving, laterally displaced apical impulse

Auscultation

• Small PDA: A murmur is sometimes heard incidentally during routine primary care visits.
• Large PDA: Machinery murmur: loud continuous murmur heard best in the left infraclavicular region; and loudest at S₂

PDA comes with Prolonged Deafening Auscultation findings.

Diagnostics ^[11][39]

• Echocardiography (confirmatory test)
  • Findings may show left atrial and ventricular dilatation
  • Used to assess shunt volume and pulmonary artery pressure
  • Color Doppler: Findings may show blood flow from the aorta into the pulmonary artery.
• ECG
  • Normal findings in small PDA
  • Left axis deviation due to LVH seen in large PDA
• Chest x-ray
  • Prominent pulmonary artery and aortic knob at the upper left heart border
  • Increased pulmonary markings
• Cardiac catheterization and angiography: only necessary prior to repair or in more complex CHDs

Treatment ^[11][12]

Management of PDA is complex and based on the specific PDA (e.g., size of left-to-right shunt) and patient characteristics (e.g., weight, age).

PDA closure ^[14]

• Indications
  • Heart failure symptoms
  • Left atrial and/or left ventricular enlargement
  • Pulmonary hypertension without right-to-left shunt
• Contraindications
  • Ductal-dependent CHD
  • Pulmonary hypertension with right-to-left shunt
• Methods
  • Transcatheter occlusion
  • Surgical ligation

Pharmacological closure in premature infants ^[3]

The management of premature infants with a PDA is complex and requires a multidisciplinary team including a neonatal intensivist and pediatric cardiologist.

• Indications
  • Infants with birth weight < 1 kg requiring mechanical ventilation
  • Infants with birth weight > 1 kg with symptomatic PDA (e.g., heart failure symptoms, respiratory distress)
• Contraindications
  • Ductal-dependent CHD
  • Persistent pulmonary hypertension of the newborn
  • Oliguria
  • Thrombocytopenia
  • Recent hemorrhage (e.g., cerebral, intestinal, pulmonary)
  • Necrotizing enterocolitis
• Treatment: Indomethacin and ibuprofen induce PDA closure by inhibiting prostaglandin synthesis. ^[3]
• Alternatives
  • Percutaneous catheter occlusion
  • Surgical ligation

PDA closure is contraindicated if the PDA is required for survival, e.g., ductal-dependent CHDs. Initiate a prostaglandin E1 infusion to keep the ductus arteriosus patent until definitive treatment can be performed. ^[40]

Complications ^[38]

• Heart failure in infancy
• Infective endocarditis
• Common cause of pulmonary hypertension and Eisenmenger syndrome in adolescents and adults
• Differential cyanosis

Definition

• Narrowing of the aorta at the aortic isthmus or, rarely, in the descending thoracic or abdominal aorta

Epidemiology ^[41]

• Prevalence: 3/10,000 live births
• Sex: ♂ > ♀

Etiology ^[2]

Congenital

• Exact cause is unknown, but two hypotheses have been proposed:
  • Hemodynamic: caused by underdevelopment of the aorta due to an abnormally decreased antegrade intrauterine blood flow
  • Ductal: caused by closure of the ductus arteriosus tissue that extends into the thoracic aorta
• Associated with Turner syndrome (in 5–15% of female patients with coarctation) ^[16][42]
• Often accompanied by a bicuspid aortic valve , VSD, and/or PDA

Acquired

• Takayasu arteritis
• Severe atherosclerosis

Pathophysiology ^[1][2]

• Genetic defects and/or intrauterine ischemia → medial thickening and intimal hyperplasia → formation of a ridge encircling the aortic lumen → narrowing of the aorta → ↑ flow proximal to the narrowing and ↓ flow distal to the narrowing
  • Coarctation is most commonly juxtaductal.
  • The coarctation most commonly occurs distal to the left subclavian artery, where the ductus arteriosus originates.
  • Rarely, the coarctation occurs in the lower segments of the thoracic aorta or in the abdominal aorta
• In discrete coarctation: left ventricular outflow obstruction → myocardial hypertrophy and increased collateral blood flow (e.g., intercostal vessels, scapular vessels).
• In long-segment coarctation: closure of PDA after birth → left ventricular pressure and volume overload → hypoperfusion of organs and extremities distal to the stenosis ^[43]

Clinical features ^[2]

General

• Neonates
  • Asymptomatic if the coarctation is mild and PDA is present
  • Symptomatic in critical stenosis (blood flow to the lower body is PDA-dependent): See “Overview”.
• May be asymptomatic in older infants, children, and adults; potential features include:
  • Differential cyanosis: cyanosis of the lower extremities
  • Brachial-femoral delay: weak femoral pulses
  • ↑ Blood pressure (BP) in upper extremities and ↓ BP in lower extremities
    • In distal narrowing of the left subclavian artery: ↑ BP in both arms and ↓ BP in both legs
    • In origin of left subclavian artery is involved: BP in the right arm > in left arm
  • Cold feet and lower-extremity claudication upon physical exertion
  • Strong apical impulse displaced to the left
  • Headache, epistaxis, tinnitus
  • In severe stenosis: shock and multiorgan failure when ductus arteriosus closes
  • In severe stenosis: See “Nonspecific findings” and “Heart failure” in “Overview” above.

Auscultation

• Systolic ejection murmur over left posterior hemithorax and/or continuous murmur below the left clavicula and between the shoulder blades

Diagnostics ^[3]

• Blood pressure measurements: (best initial test): BP measurements for upper and lower extremities to check for brachial-femoral delay
• Postcoarctation pulse oximetry: ↓ SpO₂ may be present
• ECG
  • Newborns: signs of RV hypertrophy
  • Older children: normal or signs of LV hypertrophy
• Doppler echocardiography (confirmatory test): location and extent of stenosis; detection of concurrent anomalies (VSD, PDA, bicuspid aortic valve)
• X-ray
  • Cardiomegaly and ↑ pulmonary vascular markings
  • Figure of 3 sign: the result of an hourglass-like narrowing of the aorta caused by predilatation and postdilatation of the aorta with an indentation at the site of coarctation
  • Rib notching: a radiographic sign caused by collateral circulation between the internal thoracic and intercostal arteries
    • Enlarged vessels compress the neighboring ribs, causing pressure atrophy.
    • Classically affects the inferior border of the 3^rd– 8^thribs
• MRI or CT
  • In complicated cases and in adults
  • To determine the length of coarctation and for intervention planning
• Genetic testing: for Turner syndrome ^[16]

Treatment ^[1][15]

• Neonates with critical coarctation
  • Initiate prostaglandin E1 infusion.
    • Facilitates post-coarctation organ perfusion by maintaining the patency of the ductus arteriosus until surgical repair can be performed
    • Example: alprostadil (off-label) ^[25][44]
  • Medical management of acyanotic CHDs (e.g., inotropic support, respiratory support).
• Noncritical coarctation ^[45]
  • Surgical correction: preferred in neonates ^[3]
  • Balloon angioplasty
  • Stent placement
• Follow-up: Monitor for restenosis, aortic aneurysm, and aortic dissection.

Complications

• Secondary hypertension
• Aortic dissection and rupture
• Berry aneurysm → cerebral hemorrhage ^[46]
• Heart failure
• Infective endocarditis

Description

• A valvular heart disease characterized by obstruction of blood outflow from the right ventricle into the pulmonary arteries during systole

Epidemiology

• Relatively common in the general population (∼10% of all CHDs) ^[3]
• Usually congenital (rarely acquired )

Pathophysiology

• Pulmonary valve stenosis → right ventricular outflow obstruction → pressure overload → right ventricular hypertrophy

Clinical features

• Depending on the grade of stenosis, symptoms of heart failure may occur.
• Systolic murmur heard best over the second left ICS at the sternal border
• S₂ wide splitting

Diagnostics ^[17]

• Echocardiography (confirmatory test): to assess the severity of stenosis
• Chest x-ray: may show pulmonary artery dilatation

Treatment ^[17][18]

• Neonates with critical pulmonary valve stenosis ^[3][47]
  • Maintain PDA until definitive treatment can be performed.
  • Method: prostaglandin E1 infusion, e.g. alprostadil (off-label) ^[25][44]
• Noncritical pulmonary valve stenosis
  • Repair is indicated in symptomatic patients with moderate to severe pulmonary valve stenosis.
  • Procedures include balloon valvuloplasty of the pulmonary valve (first-line) and surgical commissurotomy.

Description ^[3][48]

• A complication of acyanotic heart disease in which the shunt reverses over time and cyanotic heart disease develops
• Can occur at any age, but usually develops during late stages of CHDs

Etiology ^[3]

Eisenmenger syndrome may develop with any cardiac defect with a left-to-right shunt, but common defects include:

• Complete AVSD
• Persistent truncus arteriosus
• VSD
• PDA

Pathogenesis ^[3][48]

1. Left-to-right shunt → prolonged pulmonary hypertension → reactive constriction with permanent remodeling of pulmonary vessels → irreversible pulmonary hypertension
2. Pulmonary hypertension → RV hypertrophy → increased RV pressure
3. RV pressure exceeds LV pressure → shunt reversal (development of right-to-left shunt) → cyanosis; , digital clubbing, and polycythemia

Clinical features ^[3]

Children at risk of Eisenmenger syndrome are often minimally symptomatic; symptoms worsen with age and with increasing pulmonary resistance.

• Cyanosis
  • Central cyanosis is prominent.
  • Differential cyanosis: cyanosis in the lower extremities (seen in patients with PDA)
• Digital clubbing
• Chest pain

Diagnosis ^[3]

• Echocardiography: to assess the underlying defect, evaluate shunt direction, and estimate RV and pulmonary arterial pressures
• Cardiac catheterization: to evaluate the extent of the shunt and measure the pressure in the heart and pulmonary circulation

Treatment ^[3]

Treatment should be guided by specialists in pulmonary hypertension and pediatric cardiology; treatment includes:

• Medical management of acyanotic heart defects
• Treatment of pulmonary hypertension
• Rare cases: surgical treatment
  • Heart-lung transplant or lung transplant with correction of CHD
  • Limited by lack of donors and poor prognosis