++
A form of acyanotic congenital heart disease (CHD) in which obstruction of the left ventricular outflow tract leads to a systolic pressure gradient between the left ventricle (LV) and the aorta.
-
Aortic valve stenosis: Most common form of aortic stenosis; frequently due to bicuspid aortic valve; identified in up to 2% of adults
-
Subvalvular stenosis: Due to fibromuscular ring or shelf below the aortic valve; may be associated with malalignment ventricular septal defect (VSD) or aortic coarctation
-
Supravalvular stenosis: Least common form of aortic stenosis; may be localized or diffuse; may be associated with Williams syndrome
++
++
-
Critical aortic stenosis: High pressure gradients across the aortic valve may result in LV failure, low cardiac output, and pulmonary edema
-
Pressure gradient may increase as cardiac output increases with growth during childhood
-
Abnormal diastolic filling is due to LV hypertrophy
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CLINICAL MANIFESTATIONS
++
-
Often asymptomatic in infancy, but can present with irritability, paleness, tachycardia, tachypnea, retractions, and rales; symptoms depend on severity and location of obstruction
-
Heart failure is most common in neonates with critical disease or in adults with untreated disease
-
Ventricular arrhythmias and sudden death may occur
-
Physical examination: Early systolic ejection click at the apex; harsh ejection systolic murmur at the base radiates to the neck; palpable left ventricular lift; precordial systolic thrill at base
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-
Chest x-ray (CXR): May show cardiomegaly (evidence of LV hypertrophy) and pulmonary edema with critical obstruction
-
ECG: LV hypertrophy or LV strain in severe disease
-
Echocardiography: Defines anatomy and hemodynamic severity of the lesion
-
Cardiac catheterization: Required to establish severity and measure pressure gradient across aortic valve, and for treatment of aortic stenosis
++
-
Prostaglandin E1 (PGE1) dilates the ductus arteriosus to augment systemic output in severely ill neonates with critical obstruction
-
Inotropic support with dopamine or epinephrine may also be required to maintain adequate cardiac output both before and after the intervention
+++
Surgical or Catheter-based Interventions
++
-
Aortic valve stenosis: Percutaneous balloon aortic valvuloplasty is the preferred approach for aortic stenosis; surgical valvotomy; aortic valve replacement with pulmonary autograft or Ross procedure (preferred in infants and young children) prosthetic valve or aortic homograft
-
Subaortic stenosis: Surgical removal of fibromuscular shelf or membrane, myomectomy may be required for muscular tunnel-like obstruction (Konno procedure)
-
Supravalvular stenosis: Surgery to widen or repair stenotic segment
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A form of acyanotic CHD characterized by openings in the atrial septum at one of the following four locations:
-
Ostium secundum: Located at site of foramen ovale; accounts for 50–70% of all atrial septal defects (ASDs)
-
Ostium primum: Located low in the septum; also called incomplete atrioventricular canal defect or endocardial cushion defect, and always associated with cleft in mitral valve
-
Sinus venosus: Located high in the septum near the superior vena cava or located low in the septum near the inferior vena cava (least common); associated with anomalous pulmonary venous return
-
Coronary sinus septal defect: A portion of the coronary sinus roof is missing, causing blood to shunt from the left atrium into the coronary sinus and then into the right atrium; least common type of ASD
++
++
-
Small defects may close spontaneously
-
Magnitude of left-to-right shunt depends on size of the defect and pulmonary and systemic vascular resistance, and relative compliance of the ventricles
-
Results in right atrial and right ventricular volume overload; if unrepaired pulmonary vascular disease can develop in adulthood
+++
CLINICAL MANIFESTATIONS
++
-
Often asymptomatic in childhood and cardiac evaluation is prompted by murmur; may not present until adulthood
-
Occasionally presents in childhood with fatigue, dyspnea, respiratory infections, and congestive heart failure (CHF)
-
CHF is more common if the ASD is associated with borderline left sided structures such as mitral valve abnormalities or mild coarctation
-
Atrial dysrhythmias including fibrillation and supraventricular tachycardia are more common in adults
-
Pulmonary hypertension is more common in adults
-
Paradoxical emboli may occur
-
Physical exam: S1 is loud or normal; S2 is widely split and fixed; prominent right ventricular cardiac impulse; midsystolic pulmonary ejection murmur; diastolic murmur at left lower sternal border (LLSB) may represent flow across the tricuspid valve if pulmonary to systemic flow ratio (Qp:Qs) is greater than 2:1
++
-
CXR: Right atrial enlargement, right ventricular hypertrophy (RVH), dilated pulmonary artery, and increased pulmonary vasculature
-
ECG: Right axis deviation and RVH
-
Echocardiography: Reveals location, size, and associated anomalies
-
Cardiac catheterization: May be used to confirm presence of the defect and determine pulmonary to systemic flow ratio (Qp:Qs)
++
-
Heart failure symptoms may be managed with digoxin and diuretics (e.g., furosemide)
-
Occasional arrhythmias may require medical management
-
Secundum ASDs close spontaneously in about 40–50% of patients
+++
Surgical or Catheter-based Interventions
++
-
Usually recommended if pulmonary:systemic flow ratio (Qp:Qs) is greater than 2:1, patient is symptomatic, or ASD is moderate or large in size
-
Uncomplicated ASDs are often closed between 2 and 4 years of age
-
Defect is sutured or a patch is applied, often under cardiopulmonary bypass, though closure devices deployed by transvenous catheterization are increasingly used
+++
COARCTATION OF THE AORTA
++
A form of acyanotic CHD in which there is narrowing of the aorta, most commonly just beyond the origin of the left subclavian artery.
++
++
-
Degree of symptoms and timing of presentation depends on severity of coarctation
-
Decreased blood flow to lower extremities and lower half of the body may occur, especially after closure of the ductus arteriosus
-
LV outflow tract obstruction leads to LV hypertrophy and increased systolic pressures; eventually LV dysfunction may develop, causing low output cardiac failure and pulmonary edema
-
Extensive collateral circulation may develop in older children and young adults who are not diagnosed early in life
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CLINICAL MANIFESTATIONS
++
-
Neonates: May present in the first 3 weeks of life (especially after closure of the ductus arteriosus) with tachypnea, poor feeding, diaphoresis, CHF, cardiogenic shock, and/or decreased femoral pulses
-
Older children: Upper extremity hypertension and claudication
-
On exam: Decreased or absent femoral pulses; systolic murmur at left sternal border between third and fourth intercostal space radiating to left infrascapular area; ejection click if bicuspid aortic valve is present
++
-
Four-extremity blood pressures (BPs): Differential in BP (>10 mm Hg) between right upper extremity and lower extremities
-
CXR: Cardiomegaly and pulmonary edema is usually seen in neonates and infants, rib notching noted in children over 6 years of age
-
ECG: LV hypertrophy and possible left atrial (LA) enlargement in older children; RV hypertrophy in neonates
-
Echocardiography: Reveals segment of coarctation and associated anomalies
-
Cardiac catheterization: May be performed to delineate affected segment and potentially for treatment in older children, adolescents, and adults
-
MRI: May help define lesion and identify collateral vessels; used for serial follow-up
++
-
PGE1 maintains patency of ductus to help provide distal perfusion in severely affected neonates. Inotropic support may be required for those presenting in extremis
-
Rebound hypertension is common in the immediate postoperative period and may require antihypertensive medication, especially in older children
++
-
Timing depends on age of diagnosis, severity of disease, and related defects
-
Surgery is usually preferred for neonates and infants. Techniques include end-to-end anastomosis, patch augmentation, and subclavian flap repair. Percutaneous balloon angioplasty or stent angioplasty for native coarctation is usually reserved for recurrent coarctation or primary therapy in older children, adolescents, or adults
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ENDOCARDIAL CUSHION DEFECT
++
A form of acyanotic CHD in which there is malformation of the endocardial cushion resulting in defects in the interatrial and/or interventricular septum. Defects may be partial or incomplete (e.g., ostium primum atrial septal defect, common atrium, posterior ventricular septal defect) or complete (e.g., complete atrioventricular canal).
++
++
-
Varying degrees of left-to-right shunt result in CHF and recurrent pneumonia. The hemodynamic effect of the lesion depends on the specific location of the defect, the degree of shunting, and valvular incompetence
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CLINICAL MANIFESTATIONS
++
-
Usually present within the first few weeks of life with failure to thrive, tachypnea, tachycardia, respiratory infections, and/or heart failure
-
Untreated defects may lead to Eisenmenger syndrome (irreversible pulmonary arterial hypertension resulting from long-standing excessive pulmonary blood flow)
-
Physical exam findings depend on the extent of the lesion and may include hyperdynamic precordium with palpable thrill at LLSB; accentuated pulmonic component of S2; variable systolic murmur may be inaudible or grade 3–4/6 and holosystolic; signs of heart failure
++
Findings depend on the extent of the lesion and may include:
-
CXR: Cardiomegaly, pulmonary vascular congestion, prominent main pulmonary artery
-
ECG: Left or superior deviation of QRS axis (−40 to −150 degrees), ventricular hypertrophy (right and/or left)
-
Echocardiography: Helps define size of the defects, size of the ventricles, and anatomy of the valve
-
Cardiac catheterization: May be used to evaluate pulmonary hypertension and to look for additional VSDs
++
++
-
Definitive surgical treatment is usually recommended before 6 months of age, particularly in infants with Down syndrome
-
Palliative pulmonary artery banding is usually reserved for cases in which more definitive surgical options are challenging (e.g., prematurity)
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HYPOPLASTIC LEFT HEART SYNDROME
++
A form of cyanotic CHD characterized by underdevelopment of the LV and ascending aorta. The mitral and aortic valves are atretic or critically stenosed. Systemic circulation is dependent on a patent ductus arteriosus (PDA).
++
-
1% of CHD, more common in males
-
Most common cause of cardiac death in the first month of life
-
Chromosomal abnormalities in up to 25% of patients
++
-
Blood returning from the lungs passes through a patent foramen ovale or an ASD into the right atrium (RA) and RV
-
If a VSD is present and the aortic valve is not completely stenotic, a small amount of blood may enter the aorta directly. Otherwise, there is complete mixing of systemic and pulmonary blood, which enters the pulmonary artery and passes through a PDA into the systemic circulation, including retrograde flow in the ascending aorta
-
As the PDA closes, systemic output decreases and metabolic acidosis ensues
+++
CLINICAL MANIFESTATIONS
++
-
Most present within 48–72 hours of life as the ductus arteriosus closes
-
Cyanosis, dyspnea, poor feeding, heart failure, hepatomegaly, poor perfusion, decreased peripheral pulses, shock
-
A minority with a restrictive ASD or intact atrial septum present immediately after birth with profound cyanosis, respiratory failure, and circulatory collapse
-
On exam: Right parasternal lift; single, loud S2; soft, nonspecific systolic ejection murmur
++
-
Prenatal ultrasound: Allows for antenatal diagnosis, counseling, and time to consider treatment options; increasingly predominant method for diagnosis in current era
-
CXR: Cardiomegaly, pulmonary venous congestion, pulmonary edema
-
ECG: RV hypertrophy
-
Echocardiography: Defines anatomy
-
Cardiac catheterization: Rarely needed in the current era except for diagnosis and treatment of restrictive ASD
++
-
Without intervention, hypoplastic left heart syndrome (HLHS) is fatal within the first month of life
-
PGE1 maintains ductal patency and systemic perfusion
-
Ratio of pulmonary vascular resistance (PVR) to systemic vascular resistance (SVR) is actively managed to assure adequate oxygenation and systemic output. Generally, oxygen saturation (SaO2) greater than 70% is adequate and SaO2 greater than 90% is undesirable because it indicates pulmonary overcirculation. Supplemental oxygen is usually NOT required
++
-
Norwood Procedure: First stage in a three-staged surgical palliation of HLHS. While survival has improved, there is still a substantial risk of death during childhood
-
Hybrid: Consisting of bilateral pulmonary artery bands and stenting of the PDA is the preferred neonatal palliation in some centers
-
Orthotopic Heart Transplantation: Will be needed in many HLHS patients, though rare as a primary therapy; requires lifelong immunomodulatory therapy; shortage of available donors
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A form of cyanotic CHD resulting from a malaligned infundibular/subpulmonary septum and characterized by (1) overriding aorta; (2) right ventricular outflow tract obstruction; (3) malalignment VSD; and (4) RV hypertrophy.
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++
-
Pulmonary valve annulus has variable size and helps determine degree of RV outflow tract obstruction
-
Severity of symptoms determined by degree of RV outflow tract obstruction (related to subvalvular pulmonary stenosis) and right-to-left shunt; degree of shunt depends on PVR, SVR, and presence or absence of a PDA
-
VSD is usually large and unrestrictive
-
Mild cases may have imperceptible cyanosis (“pink tet”)
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CLINICAL MANIFESTATIONS
++
-
Paroxysmal Hypercyanotic Attacks (“Tet spells”)
-
✓ Characterized by the sudden onset of increased cyanosis, dyspnea, and change in mental status (often with irritability)
-
✓ Due to sudden increased ratio of pulmonary to SVR resulting in increased right-to-left shunting across the VSD and reduction in pulmonary blood flow
-
✓ May lead to severe hypoxemia, metabolic acidosis, and death
-
✓ Onset generally between 2 and 9 months of age
-
If untreated, cyanosis is observed in most patients by 1 year of age; dyspnea with exertion; clubbing and tendency to assume a “knee-to-chest” or squatting position are seen in older children
-
On exam: RV impulse harsh systolic ejection murmur at left sternal border; single second heart sound
++
-
CXR: “Boot-shaped” heart, clear lung fields, possible right aortic arch
-
ECG: Right axis deviation, RV hypertrophy, dominant R-wave or RSR’ pattern in precordial leads
-
Echocardiography: Defines anatomy
-
Cardiac catheterization: May rarely be required to delineate coronary artery anatomy
++
-
Remove restrictive clothing, calm patient, and place the baby in parent’s lap in knee-to-chest position
-
Oxygen
-
Morphine
-
Phenylephrine or IV beta-blocker rarely required
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-
In neonates, avoid stressors such as cold, and monitor blood glucose levels
-
If RV outflow tract obstruction is severe, infants may be dependent on a PDA and require PGE1
-
Oral propranolol may decrease frequency and severity of “Tet” spells
++
-
Palliative surgery: Systemic-to-pulmonary artery shunt (e.g., modified Blalock–Taussig shunt) can augment pulmonary blood flow in severely affected infants
-
Total surgical correction: Often done during infancy
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TOTAL ANOMALOUS PULMONARY VENOUS RETURN
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A form of cyanotic CHD in which the pulmonary veins drain anomalously into systemic veins. There are four types of total anomalous pulmonary venous return (TAPVR):
-
Supracardiac: Pulmonary veins course superiorly to a “vertical vein,” which drains into the innominate vein. Blood then flows to the superior vena cava and the RA
-
Infracardiac: Pulmonary veins course inferiorly through a descending vein, which drains into the portal system. Blood then flows to the hepatic veins, inferior vena cava, and RA. Pulmonary venous obstruction may occur at multiple levels in the descending vein
-
Cardiac: Pulmonary veins insert directly into the coronary sinus and RA
-
Mixed: Combination of other types
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++
-
Because pulmonary venous return enters systemic venous circulation, mixing through an ASD or a patent foramen ovale must occur for survival
-
Right atrial, ventricular, and pulmonary artery dilation are common due to volume overload
-
If pulmonary venous obstruction exists (common with infracardiac TAPVR), pulmonary congestion and pulmonary hypertension develop. In this case, neonates will be profoundly cyanotic and show signs of respiratory distress immediately after birth
-
If there is no pulmonary venous obstruction and the ASD is not restrictive, then oxygen saturations above 90% are common. These patients are still at risk for right heart failure
+++
CLINICAL MANIFESTATIONS
++
-
If pulmonary venous obstruction exists, patients present at 24–48 hours of life with cyanosis, tachypnea, and tachycardia
-
If pulmonary venous obstruction does not exist, patients present with mild cyanosis, failure to thrive, dyspnea, and/or CHF
-
With pulmonary venous obstruction: Single, loud S2; gallop; faint or no murmur
-
Without pulmonary venous obstruction: Increased right ventricular impulse; S2 widely split and fixed; 2/6–3/6 systolic ejection murmur at left upper sternal border; middiastolic rumble at LLSB
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-
CXR: If pulmonary venous obstruction exists, pulmonary edema is seen. If pulmonary venous obstruction does not exist, cardiomegaly is seen
-
ECG: RV hypertrophy
-
Echocardiography: Large RV, compressed LV, ASD, or patent foramen ovale
-
MRI: May be used to confirm diagnosis and help define anatomy
-
Cardiac catheterization and angiography: Helps define anatomy, ratio of pulmonary to systemic flow, and degree of pulmonary hypertension
++
-
Supplemental oxygen
-
PGE1 may decrease venous obstruction in infracardiac TAPVR by maintaining patency of the ductus venosus. However, PGE1 carries a risk of worsening pulmonary congestion by increasing left to right shunt through a PDA
-
In obstructive type of TAPVR, emergent surgery will relieve pulmonary venous obstruction. In the absence of obstruction, surgery is generally recommended in infancy
-
Goal of surgery is to redirect/connect pulmonary venous return to the LA
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TRANSPOSITION OF THE GREAT ARTERIES
++
A form of cyanotic CHD whereby the aorta arises from the morphological RV and the pulmonary artery arises from the morphological LV.
-
The most common form is dextra-transposition of the great arteries (d-TGA) in which the aorta is anterior and to the right of the pulmonary artery
-
Associated abnormalities include VSD, pulmonary stenosis, and coarctation of the aorta
++
++
-
TGA results in two parallel circuits such that deoxygenated blood is carried by the aorta to the body, while oxygenated blood is carried by the pulmonary artery to the lungs
-
To sustain life, mixing must occur through an associated PDA, VSD, or ASD, with the best mixing occurring at the level of the ASD
-
If untreated, TGA is usually fatal in the neonatal period
+++
CLINICAL MANIFESTATIONS
++
-
Cyanosis immediately after birth is typical in TGA with intact ventricular septum
-
On exam: Often, no murmur is appreciated; murmur of VSD may be noted; single, loud S2
++
-
Hyperoxia Test: 100% oxygen is administered via oxyhood for 10 minutes. If PaO2 increases above 100 mm Hg, parenchymal lung disease is suspected, whereas a PaO2 less than 50 indicates cyanotic heart disease
-
CXR: Mild cardiomegaly, “egg-on-a-string” appearance of cardiac silhouette, pulmonary vascular congestion
-
ECG: Right axis deviation, RV hypertrophy
-
Echocardiography: Confirms anatomy and associated defects; helps estimate degree of mixing
-
Cardiac catheterization: Angiogram may be used to define coronary anatomy; may be accompanied by balloon atrial septostomy as initial palliative procedure for adequate mixing
++
++
-
Balloon Atrial Septostomy (Rashkind Procedure): Increases interatrial mixing and indicated in the presence of cyanosis and a restrictive ASD
-
Arterial Switch Operation (ASO): Restores LV as the systemic pump
-
Atrial Switch Operation (Mustard or Senning technique): Risk of late RV failure and arrhythmias and rarely used in the current era
++
A form of cyanotic CHD in which there is no outlet from the RA to the RV and the RV is hypoplastic. The entire systemic blood flow enters the LA via a patent foramen ovale or an ASD.
++
++
A VSD is usually present. The pathophysiology in tricuspid atresia depends on whether the great arteries are normally related or transposed, and whether there is any obstruction to pulmonary or systemic blood flow.
+++
CLINICAL MANIFESTATIONS
++
-
In the presence of pulmonary atresia or severe pulmonary stenosis, presentation is within 24–48 hours
-
Most patients present by 2 months of age with cyanosis and tachypnea
-
Occasionally, patients with TGA develop pulmonary overcirculation and present with CHF
-
Rarely, older patients present with cyanosis, dyspnea on exertion, polycythemia, and easy fatigability
-
On exam: May have holosystolic murmur at left sternal border or ejection systolic murmur at left upper sternal border; single S2; increased LV impulse
++
-
CXR: May see pulmonary undercirculation or overcirculation
-
ECG: Left axis deviation, RA enlargement, LV hypertrophy
-
Echocardiography: Usually sufficient to delineate anatomic features
++
-
PGE1 in severely cyanotic infants (e.g., pulmonary atresia or severe pulmonic stenosis and subpulmonic stenosis) maintains patency of ductus arteriosus and promotes pulmonary blood flow
-
Treatment of CHF may be necessary in patients with high pulmonary flow
++
-
If pulmonary blood flow is diminished, initial palliative procedures may include balloon atrial septostomy, Blalock–Taussig shunt, or surgical septectomy
-
If pulmonary blood flow is increased, pulmonary arterial banding may be beneficial
-
Ultimate goal is staged palliation to Fontan completion
++
A form of cyanotic CHD in which a single arterial trunk arising from the heart supplies the coronary, pulmonary, and systemic, circulations.
++
++
-
Because blood leaves the heart through a single trunk, complete mixing occurs and cyanosis may be minimal
-
Degree of arterial SaO2 depends on the ratio of SVR to PVR
-
As PVR decreases postnatally, pulmonary blood flow increases and heart failure often develops
-
Associated anomalies may include truncal stenosis, truncal insufficiency, and interrupted aortic arch
+++
CLINICAL MANIFESTATIONS
++
-
Usually present with mild cyanosis, and tachypnea in the first month of life
-
Neonates with truncus and interrupted aortic arch present within the first 24–48 hours when the ductus arteriosus constricts
-
Features of 22q11 microdeletion/DiGeorge syndrome
-
On physical exam: Bounding pulses; systolic ejection click; single S2; harsh systolic murmur; diastolic decrescendo murmur if truncal insufficiency
++
-
CXR: Cardiomegaly, boot-shaped heart, pulmonary congestion, possible right aortic arch
-
ECG: LV hypertrophy, RV hypertrophy
-
Echocardiography: Usually sufficient to delineate anatomy
++
++
-
Patch closure of the VSD, placement of a conduit from the RV to the pulmonary arteries after separating them from the truncus
-
In the past, surgical banding of pulmonary arteries was used to limit pulmonary overcirculation; this strategy is no longer used due to the high incidence of pulmonary hypertension
+++
VENTRICULAR SEPTAL DEFECT
++
A form of acyanotic CHD characterized by an opening in the ventricular septum in one of four locations:
-
Perimembranous or conoventricular: Defect involving the membranous septum beneath the aortic valve; 70% of VSDs
-
Muscular (trabecular): Defect within the muscular septum between the LV and RV; often involves multiple, small defects which may be difficult to repair surgically; 5–20% of VSDs
-
Outlet (supracristal, subpulmonary, malalignment, conoseptal hypoplasia, subarterial): Defect beneath the pulmonic valve which communicates with the RV outflow tract; 5–7% of VSDs
-
Inlet or canal type: Located posteriorly and inferiorly to perimembranous VSDs; 5–8% of VSDs; often associated with endocardial cushion defects
++
++
-
Small defects (restrictive) are not usually hemodynamically significant
-
Large defects (unrestrictive) allow significant left-to-right shunting as the PVR declines, causing pulmonary overcirculation, and heart failure
-
Large, unrepaired defects can lead to pulmonary vascular obstructive disease and Eisenmenger’s syndrome
-
Complications include pulmonary vascular obstructive disease, RV outflow tract obstruction, aortic regurgitation, and endocarditis
+++
CLINICAL MANIFESTATIONS
++
-
May be asymptomatic and present with a murmur
-
Symptomatic VSDs often present at 4–6 weeks of age as PVR decreases and may present with dyspnea, poor growth, feeding difficulties, sweating, fatigue
-
VSDs are often silent in the newborn period while PVR is similar to SVR
-
Physical exam findings vary depending on size and location of the VSD as well as the degree of PVR and may include loud, harsh, blowing holosystolic murmur at LLSB; palpable thrill at LLSB with parasternal lift and apical thrust; S3 and a middiastolic rumble may be present. Small defects may be associated with loud murmurs
++
-
CXR: May be normal or may reveal cardiomegaly and increased pulmonary vasculature
-
ECG: May be normal or may reveal evidence of LV hypertrophy, LA hypertrophy, and biventricular hypertrophy
-
Echocardiography: Reveals the size and location of the VSD
-
Cardiac catheterization: Rarely indicated in the current era
++
-
Small VSDs are often well tolerated
-
Approximately 70% of VSDs close spontaneously. Small, muscular defects are most likely to close spontaneously
-
If signs of heart failure, consider diuretics, ACE inhibitors, or digoxin
++
-
Indications for surgery include uncontrolled heart failure, development of aortic regurgitation, RV outflow tract obstruction
-
Surgical closure with a Dacron or Gortex patch
-
Pulmonary arterial palliative banding is usually reserved for complicated cases and premature infants
+++
SURGERIES FOR CONGENITAL HEART DISEASE
+++
ARTERIAL SWITCH OPERATION (OF JATENE)
++
-
Indication: Transposition of the great arteries (TGA)
-
Definition: The coronary arteries are reimplanted into the pulmonary artery (neoaorta). The pulmonary artery and aorta are transected above the sinus of Valsalva, the pulmonary artery is usually brought in front of the neo-aorta (Lecompte maneuver), and reattached
+++
BLALOCK–TAUSSIG SHUNT
++
-
Indication: Tetralogy of Fallot, tricuspid atresia, pulmonary atresia
-
Definition: Direct anastomosis of the subclavian artery to the ipsilateral pulmonary artery, thereby creating a systemic to pulmonary shunt
+++
BLALOCK–TAUSSIG SHUNT, MODIFIED
++
-
Indication: Tetralogy of Fallot, tricuspid atresia, pulmonary atresia
-
Definition: A Gortex graft connects the subclavian artery to the ipsilateral pulmonary artery, thereby creating a systemic to pulmonary shunt
+++
NORWOOD PROCEDURE OR STAGE I
++
-
Indication: Usually first stage for single ventricle heart disease (e.g., HLHS and variants)
-
Reconstruction of the hypoplastic ascending aorta and the aortic arch using the main pulmonary artery and homograft patch, placement of a Blalock–Taussig shunt or shunt from RV to the pulmonary artery (Sano modification), and removal of the atrial septum
+++
BIDIRECTIONAL GLENN SHUNT OR HEMI-FONTAN (4–6 MONTHS)
++
-
Indication: Usually second stage for single ventricle heart disease (e.g., tricuspid atresia, HLHS)
-
Definition: Direct connection of the superior vena cava to undivided right pulmonary artery, allowing blood flow to both lungs
+++
FONTAN PROCEDURE (2–4 YEARS)
++
-
Indication: Usually third stage for single ventricle heart disease
-
Definition: Connection of the inferior vena cava to the right pulmonary artery. Modifications include lateral tunnel and extracardiac conduit with or without fenestration
+++
MUSTARD AND SENNING PROCEDURE
++
+++
PULMONARY ARTERY BANDING
++
-
Indication: Single ventricle heart disease with increased pulmonary blood flow, complicated VSD
-
Definition: Constriction of the pulmonary artery to reduce pulmonary blood flow
++
-
Indication: Aortic stenosis, aortic regurgitation, or mixed aortic valve disease
-
Definition: Replacement of diseased aortic valve and root with patient’s own pulmonary valve and root (autograft). A homograft is placed into the position of the pulmonary valve, and the coronary arteries are reimplanted into the autograft