WORKUP

Lab Studies:

• Complete blood count

• Obtain hemoglobin because severe neonatal anemia can cause high-output congestive heart failure (CHF) and cardiogenic shock. The hemoglobin is usually normal.

• Obtain a total white blood cell (WBC) count with differential. Sepsis can cause symptoms of shock. The WBC count is typically normal.

• Electrolytes

• Abnormalities may be present in infants with poor oral intake secondary to CHF. Use carbon dioxide to assess acid-base status.

• Electrolytes are usually normal. The carbon dioxide level may be low if a metabolic acidosis is present.

• BUN/creatinine

• Infants with critical illness and significantly reduced systemic perfusion may show evidence of renal failure.

• The creatinine may be elevated transiently.

• Liver function tests

• Infants with critical illness and significantly reduced systemic perfusion and CHF may show evidence of hepatocellular damage.

• Serum glutamic-oxaloacetic transaminase (SGOT) and serum glutamic-pyruvic transaminase (SGPT) may be elevated transiently.

• Arterial blood gases and lactic acid

• Assessing acid-base status is paramount, especially to rule out metabolic acidosis. Most infants have some evidence of metabolic acidosis, which should be corrected immediately. Serum lactic acid elevation generally precedes fall in pH as acidosis develops.

• Assessment of PaO₂ and PaCO₂ is important for respiratory management and manipulation of pulmonary vascular resistance by mechanical ventilation and the addition of supplemental inhaled nitrogen. The PaO₂ is optimally between 30-45 mmHg, and the PaCO₂ is ideally between 45-50 mm Hg.

• Ultrasound of the head

• An ultrasound of the head is necessary only if the infant has had a significantly long period in shock with potentially poor cerebral perfusion.

• Most often, no abnormalities are observed on the ultrasound scan of the head.

• Karyotype

• Chromosomal analysis is indicated for infants with dysmorphic features.

• Nearly 25% of infants have chromosomal abnormalities.

Imaging Studies:

• Chest x-ray

• These findings are not specific for the condition.

• Cardiomegaly and increased pulmonary venous-vascular markings typically are present.

• Marked pulmonary edema may be noted in infants with obstructed pulmonary venous return.

• Echocardiogram

• The echocardiogram is the test of choice for diagnosing HLHS. Two-dimensional imaging clearly shows the hypoplastic left ventricle and ascending aorta. The right atrium, tricuspid valve, right ventricle, and main pulmonary artery are larger than usual.

• Other structural abnormalities should be excluded.

• Doppler and color Doppler imaging are also important.

• Evaluate tricuspid regurgitation, a preoperative risk factor for the Norwood procedure, and blood flow across the atrial septum. Observe retrograde blood flow from the ductus arteriosus into the transverse aortic arch and ascending aorta.

• Evaluate the aortic arch and thoracic aorta for evidence of coarctation.

Other Tests:

• Electrocardiogram

• This typically shows sinus tachycardia, right axis deviation, right atrial enlargement, and right ventricular hypertrophy with a qR pattern in the right precordial leads.

• A paucity of left ventricular forces is noted in the left precordial leads.

Procedures:

• Cardiac catheterization

• Pre-Norwood procedure
  • Routine diagnostic catheterization is not necessary because 2-dimensional and Doppler echocardiography can provide the necessary anatomic and hemodynamic data..

  • Perform interventional catheterization with blade/balloon atrial septostomy to relieve pulmonary venous hypertension if blood flow from left atrium to right atrium is severely restricted at the atrial septum.

• Pre-hemi-Fontan (stage II) procedure
  • Perform routine catheterization before the operation to obtain hemodynamic data and several important angiograms.

  • Calculate pulmonary vascular resistance to assure the patient's suitability for stage II.

  • Perform an angiogram in the right ventricle to show ventricular function and tricuspid regurgitation.

  • Perform another angiogram in the transverse aortic arch near the shunt to show pulmonary artery size and distribution and to rule-out recurrent aortic coarctation or significant aortopulmonary collateral vessels.

  • If collateral vessels are found, they may be occluded with coils at the same catheterization.

• Pre-Fontan (stage III) procedure
  • Accomplish routine catheterization before completing the operation.

  • Calculate pulmonary vascular resistance and perform a right ventricular angiogram.

  • Delineate pulmonary artery anatomy by performing an angiogram at the superior vena cava–pulmonary artery anastomosis via an internal jugular approach.

  • Recurrent coarctation of the aorta and significant collateral vessels are excluded again.

• Postcatheterization precautions include hemorrhage, vascular disruption after balloon dilation, pain, nausea and vomiting, and arterial or venous obstruction from thrombosis or spasm.

TREATMENT

Medical Care:

• Successful preoperative management depends on providing adequate systemic blood flow while limiting pulmonary overcirculation.

• Open the ductus arteriosus
  • Blood flow to the systemic circulation (coronary arteries, brain, liver, kidneys) is dependent on flow through the ductus arteriosus. If a diagnosis is suspected, start prostaglandin (PGE) infusion immediately to establish ductal patency and ensure adequate systemic perfusion.

  • If the diagnosis is made prenatally or when the infant is relatively asymptomatic, a smaller dose of PGE may be sufficient to keep the ductus arteriosus patent while limiting its side effects.

  • A larger dose of PGE often is required to reopen the ductus arteriosus if an infant has cardiovascular collapse and shock due to ductal closure.

  • Ideally, PGE is administered centrally via an umbilical venous catheter.

• Correct metabolic acidosis
  • Metabolic acidosis indicates inadequate cardiac output to meet the metabolic demands of the body. Acidosis adversely affects the myocardium.

  • Correction of metabolic acidosis with sodium bicarbonate infusion is essential in early management. This therapy is futile if the ductus arteriosus remains constricted.

• Manipulate pulmonary vascular resistance
  • The pulmonary vascular resistance of a newborn is slightly less than the systemic vascular resistance and begins to fall soon after birth. In the patient with HLHS, decreased pulmonary vascular resistance causes increased pulmonary blood flow and an undesirable obligatory decrease in systemic blood flow. Increased alveolar oxygen decreases pulmonary vascular resistance, leading to increased pulmonary blood flow.

  • Therefore, most infants should remain in room air with acceptable oxygen saturation (pulse oximeter) in the low 70s. An exceptional circumstance would be in the infant with severe hypoxemia caused by pulmonary venous hypertension.

  • Achieving a slightly higher PaCO₂, in the range of 45-50 mm Hg, can increase pulmonary vascular resistance. This can be accomplished by intubation, sedation, mechanical hypoventilation, or by the addition of nitrogen or carbon dioxide to the infant's inspired gas via the endotracheal tube or hood. It is preferable not to intubate these infants.

  • Serial blood gas analysis is necessary. Initially, an umbilical arterial catheter is useful to obtain frequent blood samples.

• Inotropes
  • Inotropic support is indicated only in severely ill neonates with concurrent sepsis or profound cardiogenic shock and acidosis.

  • The administration of inotropes can adversely affect the balance between pulmonary and systemic vascular resistance.

  • If needed, wean from inotropic support as soon as the infant is clinically stable.

• Diuretics
  • Consider diuretics to manage pulmonary overcirculation before surgery.

  • Agents commonly used include furosemide and spironolactone.

• Antibiotics
  • Antibiotics are indicated if the infant is at risk for antepartum infection.

  • Discontinue antibiotics after obtaining negative blood cultures.

Surgical Care:

• The goal of surgical reconstruction is eventually to separate the pulmonary and systemic circulations by completing a Fontan operation. The right ventricle remains the systemic ventricle while blood flows to the lungs passively. This ultimate reconstruction is accomplished in 3 stages.

• Norwood procedure (stage I)
  • This is usually performed during the first weeks of life, after the infant has been stabilized in the neonatal intensive care unit (ICU). The goals of the procedure are (1) to establish reliable systemic circulation in the absence of the ductus arteriosus and (2) to provide enough pulmonary blood flow for adequate oxygenation, while simultaneously protecting the pulmonary vascular bed in preparation for stages II and III.

  • The Norwood procedure includes (1) performing an atrial septectomy to provide unrestricted blood flow across the atrial septum, (2) ligating the ductus arteriosus, (3) creating an anastomosis between the main pulmonary artery and aorta to provide systemic blood flow, (4) eliminating coarctation of the aorta, and (5) placing an aorta–to–pulmonary artery shunt to provide pulmonary circulation.

  • At hospital discharge, most infants remain on digoxin to augment cardiac function, diuretics to help manage right ventricular volume overload, and aspirin to prevent thrombosis of the shunt. If tricuspid regurgitation is present, use afterload reduction with captopril. Oxygen saturation is typically 70-80% in room air.

• Hemi-Fontan procedure (stage II)
  • This is performed approximately 6 months after the Norwood procedure. Before surgery, perform a cardiac catheterization to assess right ventricular function, pulmonary artery anatomy, and pulmonary vascular resistance. If results are favorable, schedule elective surgery.

  • The hemi-Fontan includes creating an anastomosis between the superior vena cava and the right pulmonary artery, so that venous return from the upper body can flow directly into both lungs. The superior vena cava-right atrial junction is closed with a patch that is removed during the next stage. Blood from the inferior vena cava continues to drain into the right atrium. The aorta–to–pulmonary artery shunt that was placed at stage I is ligated.

  • At discharge, infants usually remain on digoxin, diuretics, aspirin, and captopril for the reasons mentioned above.

• Fontan procedure (stage III)
  • This occurs approximately 12 months after the hemi-Fontan procedure. Again, catheterization is necessary to ensure that the child is a candidate for surgery.

  • Completion of the Fontan procedure includes directing blood flow from the inferior vena cava to the pulmonary arteries by placing a tube within the right atrium. At the conclusion of the procedure, systemic venous blood returns to the lungs passively without passing through a ventricle.

  • At discharge, most children remain on digoxin, diuretics, aspirin, and captopril if necessary. In an uncomplicated case, most of these medications can be weaned over the 6 months following the Fontan operation. Some advocate using aspirin indefinitely.

• Orthotopic cardiac transplantation

• Heart transplantation is another surgical option. The infant must remain on PGE infusion to keep the ductus arteriosus patent while waiting for a donor heart to become available. Approximately 20% of infants listed for heart transplantation die waiting for a suitable donor organ.

• After successful cardiac transplantation, infants require multiple medications for modulation of the immune system and prevention of graft rejection. Perform frequent outpatient surveillance to identify rejection early and prevent lasting damage to the transplanted heart. Periodic endomyocardial biopsy usually is performed for more precise monitoring.

Consultations:

• Consult a pediatric cardiologist.

• Consult a pediatric cardiovascular surgeon

• Consult a genetic specialist if a chromosomal abnormality is suspected.

Diet:

• Adequate nutrition is important before and after surgery. Many infants require nasogastric feeding with increased calorie breast milk or formula after the Norwood procedure. However, normal oral feeding is reestablished with time. Adequate oral iron intake prevents development of iron deficiency anemia.

• After completion of the Fontan operation, specific dietary restrictions are not necessary.

Activity:

• Specific activity restrictions are not imposed on children after completion of the Fontan operation. In general, encourage children to participate in activities that they are able to tolerate.

• Studies have shown that these children may have impaired exercise performance when compared to age-matched peers. Perform an exercise stress test when the child is old enough.

• Neurodevelopmental abnormalities occur often in patients with HLHS.

MEDICATION

Before the Norwood procedure or cardiac transplantation, treat infants with prostaglandin infusion, diuretics, inotropes, and afterload reduction. The medical management after cardiac transplantation is not discussed in this article.

Drug Category: Prostaglandins -- PGE-1 promotes dilatation of the ductus arteriosus in infants with ductal-dependent cardiac abnormalities.

Drug Name	Alprostadil (Prostaglandin E1, Prostin) -- Causes relaxation of smooth muscle, primarily within the ductus arteriosus. Used in infants with ductal-dependent congenital heart disease due to restricted systemic blood flow.
Pediatric Dose	0.01-0.1 mcg/kg/min IV infusion
Contraindications	Documented hypersensitivity; respiratory distress syndrome or persistent fetal circulation
Interactions	Coadministration with heparin may increase PTT or PT
Pregnancy	X - Contraindicated in pregnancy
Precautions	Closely monitor respiratory, cardiovascular status, and coagulation; apnea, fever, irritability, and cutaneous flushing are common; inhibits platelet aggregation

Drug Category: Diuretic agents -- Decreases preload by increasing free water excretion. Decreasing preload may improve systolic ventricular function.

Drug Name	Furosemide (Lasix) -- Loop diuretic that blocks sodium reabsorption in the ascending loop of Henle.
Adult Dose	20-80 mg IV/IM/PO up to tid
Pediatric Dose	0.5-2 mg/kg IV/IM/PO up to tid
Contraindications	Documented hypersensitivity; hepatic coma, anuria, and severe electrolyte depletion
Interactions	Antagonizes muscle relaxing effect of tubocurarine; auditory toxicity appears to be increased with coadministration of aminoglycosides and furosemide; hearing loss of varying degrees may occur; anticoagulant activity of warfarin may be enhanced when taken concurrently with this medication
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Profound diuresis and electrolyte loss may result; metabolic alkalosis; use caution with other medications known to decrease renal function; may cause hypercalciuria and renal stones, especially in premature infants

Drug Name	Spironolactone (Aldactone) -- This drug is a potassium-sparing loop diuretic.
Adult Dose	25-100 mg PO divided bid/qid
Pediatric Dose	2-3 mg/kg PO qd or divided bid
Contraindications	Documented hypersensitivity; anuria, renal failure or hyperkalemia
Interactions	May decrease effect of anticoagulants; potassium and potassium sparing diuretics may increase toxicity of spironolactone
Pregnancy	D - Unsafe in pregnancy
Precautions	Electrolyte imbalance, especially hyperkalemia, may result; concomitant use with indomethacin or ACE inhibitors may cause hyperkalemia

Drug Category: Cardiac glycosides -- These medications improve ventricular systolic function by increasing the calcium supply available for myocyte contraction.

Drug Name	Digoxin (Lanoxin) -- This form inhibits the sodium-potassium ATPase pump in cardiac myocytes.
Adult Dose	Total digitalizing dose (TDD): 1-1.5 mg PO given in divided doses over 1 d Maintenance dose: 0.125-0.375 mg PO in 1-2 doses
Pediatric Dose	TDD: Premature infants: 0.02 mg/kg PO divided q8h for 3 doses Full-term infants: 0.03 mg/kg PO divided q8h for 3 doses 1-24 months: 0.04-0.05 mg/kg PO divided q8h for 3 doses >2 years: 0.03-0.04 mg/kg PO divided q8h for 3 doses Maintenance dose: Infants: 6-8 mcg/kg/d PO 2-5 years: 10-15 mcg/kg/d PO 5-10 years: 7 to 10 mcg/kg/d PO >10 years: 3-5 mcg/kg/d PO <10 years: bid dosing recommended
Contraindications	Documented hypersensitivity; beriberi heart disease, idiopathic hypertrophic subaortic stenosis, constrictive pericarditis, and carotid sinus syndrome
Interactions	Medications that may increase digoxin levels include alprazolam, benzodiazepines, bepridil, captopril, cyclosporine, propafenone, propantheline, quinidine, diltiazem, aminoglycosides, oral amiodarone, anticholinergics, diphenoxylate, erythromycin, felodipine, flecainide, hydroxychloroquine, itraconazole, nifedipine, omeprazole, quinine, ibuprofen, indomethacin, esmolol, tetracycline, tolbutamide, and verapamil Medications that may decrease serum digoxin levels include aminoglutethimide, antihistamines, cholestyramine, neomycin, penicillamine, aminoglycosides, oral colestipol, hydantoins, hypoglycemic agents, antineoplastic treatment combinations (including carmustine, bleomycin, methotrexate, cytarabine, doxorubicin, cyclophosphamide, vincristine, procarbazine), aluminum or magnesium antacids, rifampin, sucralfate, sulfasalazine, barbiturates, kaolin/pectin, and aminosalicylic acid
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Hypokalemia may reduce positive inotropic effect of digitalis; IV calcium may produce arrhythmias in digitalized patients; hypercalcemia predisposes patient to digitalis toxicity, and hypocalcemia can make digoxin ineffective until serum calcium levels are normal; magnesium replacement therapy must be instituted in patients with hypomagnesemia to prevent digitalis toxicity; patients with incomplete AV block may progress to complete block when treated with digoxin; exercise caution in hypothyroidism, hypoxia, and acute myocarditis

Drug Category: Inotropic agents -- These agents stimulate alpha- and beta-receptors in the heart and vascular bed.

Drug Name	Dopamine (Intropin) -- At lower doses, stimulation of beta1-adrenergic and beta1-dopaminergic receptors results in positive inotropism and renal vasodilatation; at higher doses, stimulation of alpha-adrenergic receptors results in peripheral and renal vasoconstriction.
Adult Dose	2-20 mcg/kg/min IV infusion
Pediatric Dose	Administer as in adults
Contraindications	Documented hypersensitivity; pheochromocytoma or ventricular fibrillation
Interactions	Phenytoin, alpha- and beta-adrenergic blockers, general anesthesia, and MAOIs increase and prolong effects of dopamine
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Use caution with intravascular volume depletion; administration via a central venous catheter is recommended; the umbilical artery should not be used; doses higher than 20 mcg/kg/min generally are not helpful and other agents should be considered; subcutaneous infiltration may cause tissue sloughing; prompt treatment with subcutaneous phentolamine (Regitine) is recommended

Drug Name	Dobutamine (Dobutrex) -- This drug primarily stimulates the beta1-adrenergic receptor and has less alpha-adrenergic stimulation leading primarily to increased myocardial contractility.
Adult Dose	2-20 mcg/kg/min IV infusion
Pediatric Dose	Administer as in adults
Contraindications	Documented hypersensitivity; idiopathic hypertrophic subaortic stenosis and atrial fibrillation or flutter
Interactions	Beta-adrenergic blockers antagonize effects of dobutamine; general anesthetics may increase toxicity
Pregnancy	B - Usually safe but benefits must outweigh the risks.
Precautions	Use caution with intravascular volume depletion; administration via a central venous catheter is recommended; the umbilical artery should not be used; doses higher than 20 mcg/kg/min generally are not helpful and other agents should be considered; subcutaneous infiltration may cause tissue ischemia

Drug Category: Afterload-reducing agents -- Afterload reduction improves myocardial performance and theoretically reduces atrioventricular and semilunar valve insufficiency.

Drug Name	Captopril (Capoten) -- ACE inhibitor, which decreases the production of angiotensin II, a potent vasoconstrictor, resulting in peripheral vasodilatation and afterload reduction, improving myocardial performance and theoretically reducing AV and semilunar valve insufficiency. Administer a test dose of 0.1 mg PO to assess initial response
Adult Dose	6.25-12.5 mg PO tid; not to exceed 150 mg tid
Pediatric Dose	0.1-1 mg/kg PO tid
Contraindications	Documented hypersensitivity; renal impairment
Interactions	NSAIDs may reduce hypotensive effects of captopril; ACE inhibitors may increase digoxin, lithium, and allopurinol levels; rifampin decreases captopril levels; probenecid may increase captopril levels; the hypotensive effects of ACE inhibitors may be enhanced when given concurrently with diuretics
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Pregnancy category D in second and third trimesters; caution in renal impairment, valvular stenosis, or severe congestive heart failure; profound hypotensive response is observed rarely after the initial dose in smaller children; an initial test dose should be given and blood pressure should be monitored carefully; dose should be titrated based on clinical response and tolerance; use caution with decreased renal function; ACE inhibitors have a potassium sparing effect when administered with furosemide; simultaneous administration of spironolactone should be done with caution

Drug Category: Antiplatelet agents -- These agents are used in the treatment or prevention of thrombo-occlusive disease mediated by the action of platelets. They inhibit platelet function by blocking cyclooxygenase and subsequent aggregation.

Drug Name	Aspirin (Anacin, Ascriptin, Bayer Aspirin) -- Inhibits the enzyme cyclooxygenase that reduces production of thromboxane A2, which is a potent vasoconstrictor and platelet-aggregating agent. Antiplatelet effects of aspirin last the entire life of the platelet (6-10 d) and are not reversible.
Adult Dose	325 mg PO qd
Pediatric Dose	5-10 mg/kg PO qd
Contraindications	Documented hypersensitivity; liver damage, hypoprothrombinemia, vitamin K deficiency, bleeding disorders, asthma; because of association of aspirin with Reye syndrome, do not use in children (<16 y) with flu
Interactions	Effects may decrease with antacids and urinary alkalinizers; corticosteroids decrease salicylate serum levels; additive hypoprothrombinemic effects and increased bleeding time may occur with coadministration of anticoagulants; may antagonize uricosuric effects of probenecid and increase toxicity of phenytoin and valproic acid; doses >2 g/d may potentiate glucose lowering effect of sulfonylurea drugs
Pregnancy	D - Unsafe in pregnancy
Precautions	May cause transient decrease in renal function and aggravate chronic kidney disease; avoid use in patients with severe anemia, with history of blood coagulation defects, or taking anticoagulants

FOLLOW-UP

Further Inpatient Care:

• Initial preoperative management and postoperative care takes place in the neonatal, pediatric, or cardiac ICUs.

• When clinically stable, transfer postoperative patients to the general cardiac unit for adjusting oral medications, addressing feeding issues, and completing discharge teaching.

• Involve a pediatric cardiologist during any noncardiac hospital admission of a patient who is status post (S/P) Norwood procedure. This is because of the complex cardiovascular physiology in infants after this surgery.

Further Outpatient Care:

• Schedule outpatient follow-up care 2 weeks after discharge in the typical postoperative patient.

• Schedule those who are S/P cardiac transplantation earlier for necessary laboratory studies.

• Earlier follow-up care is also necessary if a pericardial effusion is discovered on the discharge echocardiogram.

• Individualize further outpatient follow-up care based on the needs of each patient.

In/Out Patient Meds:

• Inpatient medications

• Prostaglandin E1

• Dopamine/dobutamine

• Furosemide (Lasix/Aldactone)

• Captopril

• Digoxin

• Outpatient medications

• Furosemide (Lasix/Aldactone)

• Captopril

• Digoxin

Transfer:

• Transfer the infant to a hospital with appropriate ICUs. Pediatric cardiology and cardiovascular surgery services must be immediately available.

• Carefully monitor the infant for apnea during transfer while on prostaglandin therapy. If PGE has been started, consider elective endotracheal intubation before transfer.

Complications:

• Preoperative complications include acidosis, CHF, renal failure, liver failure, necrotizing enterocolitis, sepsis, and death.

• Postoperative complications include acidosis, CHF, renal failure, liver failure, necrotizing enterocolitis, sepsis, pericardial or pleural effusion, phrenic or recurrent laryngeal nerve damage, stroke, coarctation of the aorta, and death. Early graft rejection and opportunist infection may occur after cardiac transplantation.

• Major complications following the Norwood procedure include aortic arch obstruction at the site of surgical anastomosis and progressive cyanosis caused by limited blood flow through the shunt. An inadequate atrial communication contributes to progressive cyanosis.

• Major complications following the hemi-Fontan procedure include transient superior vena cava syndrome and persistent pleural or pericardial effusion. The development of systemic venous to pulmonary venous collateral vessels is possible.

• Major complications following the Fontan procedure include persistent pleural or pericardial effusion. Neurodevelopmental abnormalities are reported and may be inherent in some patients with HLHS.

Prognosis:

• Overall survival to the time of hospital discharge after the Norwood procedure is nearly 75%. Success rates are higher in uncomplicated cases and lower in patients who have important preoperative risk factors, including operation in patients older than 1 month, significant preoperative tricuspid insufficiency, pulmonary venous hypertension, associated major chromosomal or noncardiac abnormalities, and prematurity.

• Survival after the hemi-Fontan and Fontan operations is nearly 90-95%.

• The actuarial survival rate after staged reconstruction is 70% at 5 years.

• Institutional success rates vary.

• Neurodevelopmental prognosis is not known; however, abnormalities are reported.

• Approximately 20% of infants listed for cardiac transplantation die while waiting for a donor heart. After successful transplantation, the survival rate at 5 years is approximately 80%.

• When the preoperative mortality is considered, the overall survival rate after cardiac transplantation is approximately 70% or similar, to the results for staged reconstruction.

Patient Education:

• Medication

• Educate parents regarding the doses and side effects of their child's cardiac medications.

• Discuss interactions with other medications with the family and the infant's general pediatrician.

• Feeding

• Many infants require nasogastric tube feeding after discharge from the hospital. Parents must become comfortable with placement of the nasogastric feeding tube.

• Frequently, increased calorie formula is required for adequate growth. Provide the formula recipe or a source for purchasing it to the caregiver.

• Follow-up care

• Stress the importance of follow-up care. If necessary, provide cab or bus vouchers to ensure compliance.

• If noncompliance becomes a critical issue, physicians are required to report to the appropriate family services agency.

MISCELLANEOUS

Medical/Legal Pitfalls:

• Misdiagnosis

• Failure to recognize the infant with HLHS and the obstruction to pulmonary venous return

Special Concerns:

• The newborn with HLHS dies rapidly if untreated. Surgical techniques, both reconstruction and heart transplantation, offer an opportunity to preserve the newborn's life. Survival rates given above represent the best results and reflect only survival, not quality of life. Mortality rates in many centers exceed those mentioned. Incidence of neurodevelopmental abnormalities in HLHS appears to exceed that of other single ventricle conditions.

• HLHS affects family structure. For example, reproductive studies indicate that incidence of subsequent pregnancy is significantly lower in mothers of a living patient with HLHS than in mothers after death of an infant with HLHS. For these reasons, most pediatric cardiologists continue to offer no treatment as an acceptable option to parents of a newborn with HLHS. It is incumbent on physicians caring for a newborn with HLHS to clearly communicate all of this information to the parents. An ethically appropriate consent for surgery requires this. Allowing an affected infant to die without surgical intervention is a difficult decision, but it is still chosen by some families.

PICTURES

 

Caption: Picture 1. This echocardiographic still frame shows a long-axis view of the aortic arch in a patient with hypoplastic left heart syndrome. The ascending aorta is markedly hypoplastic, serving only to deliver blood in a retrograde fashion to the coronary arteries. An echo-bright coarctation shelf is seen at the insertion of the ductus arteriosus.

Picture Type: Photo

Caption: Picture 2. This echocardiographic still frame shows a 4-chamber view of the heart in a patient with hypoplastic left heart syndrome. A large right ventricle (RV) and hypoplastic left ventricle (star) are seen. RA, right atrium; LA, left atrium.

Picture Type: Photo