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  1. 1. CASE PRESENTATION <ul><li>This is a two-day old male infant, born weighting 2730 grams (six pounds, two ounces) after a full-term pregnancy complicated by influenza in the first trimester, was found to be tachypneic on the second day of life. Examination at that time also revealed weak peripheral pulses. He was transferred to the university hospital. </li></ul>
  2. 2. CASE PRESENTATION (cont.) <ul><li>On admission, physical examination revealed a small tachypneic infant with grayish cyanosis of the lips. The respiratory rate was 89 per minute and the heart rate 170 per minute. The lungs were clear to auscultation. A right ventricular lift was noted at the left sternal border and in the epigastric area. No thrills were present. </li></ul>
  3. 3. CASE PRESENTATION (cont.) <ul><li>The first heart sound was normal but the second sound was closely split with a loud, snappy pulmonic component. A short, soft ejection systolic murmur was present at the fourth left intercostal space. The peripheral pulses could not be palpated in either the upper or lower extremities. The liver edge was palpable 3-4 cm below the RCM. </li></ul>
  4. 4. QUESTIONS <ul><li>The differential diagnosis of congestive heart failure in the first few days of life include all the following except : </li></ul><ul><li>A. TAPVR </li></ul><ul><li>B. Myocarditis </li></ul><ul><li>C. Hypoplastic left heart syndrome </li></ul><ul><li>D. Preductal coarctation of the aorta </li></ul><ul><li>E. VSD </li></ul>
  5. 5. QUESTIONS (cont.) <ul><li>Anatomical components of the hypoplastic heart syndrome include all the following except: </li></ul><ul><ul><li>A. Aortic atresia </li></ul></ul><ul><ul><li>B. Minute left ventricle </li></ul></ul><ul><ul><li>C. Mitral atresia, stenosis or hypoplasia </li></ul></ul><ul><ul><li>D. PDA </li></ul></ul><ul><ul><li>E. Pulmonary atresia </li></ul></ul>
  6. 6. QUESTIONS (cont.) <ul><li>3. Which of the following findings is not a typical one in patients with HLHS? </li></ul><ul><li>A. Fairly sudden onset of tachypnea and dyspnea during the first week of life. </li></ul><ul><li>B. Loud aortic component of the S2. </li></ul><ul><li>C. Loud pulmonary component of the S2. </li></ul><ul><li>D. Weak peripheral pulses. </li></ul><ul><li>E. Cyanosis </li></ul>
  7. 7. HYPOPLASTIC LEFT HEART SYNDROME Gerardo Gonzalez, MD
  8. 8. HLHS - History <ul><li>First described in 1952 by Lev as the pathologic complex “ hypoplasia of the aortic tract”, including cases of: </li></ul><ul><ul><li>isolated hypoplasia of the aorta </li></ul></ul><ul><ul><li>hypoplasia of the aorta and VSD </li></ul></ul><ul><ul><li>hypoplasia of the aorta with aortic stenosis or atresia, with or without mitral stenosis or atresia </li></ul></ul><ul><li>In 1958, Noonan and Nadas termed these lesions as “hypoplastic left heart syndrome”. </li></ul>
  9. 9. HLHS - Embryology <ul><li>The embryologic cause is not fully understood. </li></ul><ul><li>It probably results from a limitation of either LV inflow or outflow, such as the development of severe AS early. </li></ul>
  10. 10. HLHS - Genetics <ul><li>Familial inheritance: </li></ul><ul><ul><li>Autosomal recessive and multifactorial inheritance have both been postulated. </li></ul></ul><ul><ul><li>Sibling recurrence risk: 0.5% </li></ul></ul><ul><ul><li>Sibling recurrence for all other cardiac malformations: 2.2% </li></ul></ul><ul><li>Definable genetic disorder (28%): </li></ul><ul><ul><li>Turner Syndrome </li></ul></ul><ul><ul><li>Noonan Syndrome </li></ul></ul><ul><ul><li>Trisomy 13, 18, 21, or other microdeletion syndromes </li></ul></ul>
  11. 11. HLHS - Epidemiology <ul><li>Each year, approximately 1000 infants with HLHS are born in the US. </li></ul><ul><li>Prevalence: 0.016-0.27 per 1000 live births. </li></ul><ul><li>In pathologic series, it accounts for 1.4-3.8 of congenital heart disease. </li></ul><ul><li>Third cause of critical CHD in the newborn. </li></ul><ul><li>Male predominance: 57-70%. </li></ul><ul><li>23% of all neonatal mortality from CHD. </li></ul>
  12. 12. HLHS – Epidemiology (cont.) <ul><li>Infants with HLHS are usually full term; they have a normal birth weight and few significant extracardiac malformations. </li></ul><ul><li>Prematurity and SGA at birth are observed in 5.5%. </li></ul><ul><li>Noncardiac malformations are uncommon (12%). </li></ul><ul><li>Serious or major malformations are rare (2.3%) </li></ul>
  13. 13. Anatomy
  14. 14. HLHS - Pathophysiology <ul><li>The term HLHS is used to describe a related group of anomalies that include underdevelopment of the left side of the heart (e.g. atresia of the aortic or mitral orifice) and hypoplasia of the ascending aorta . </li></ul><ul><li>The left ventricle may be small and nonfunctional or totally atretic. </li></ul>
  15. 15. HLHS - Pathophysiology <ul><li>The right ventricle maintains both pulmonary and systemic circulations. </li></ul><ul><li>Pulmonary venous blood passed through an atrial defect or dilated foramen ovale from the left to the right side of the heart, where it mixes with systemic venous blood ( TOTAL MIXING LESION ). </li></ul><ul><li>The ventricular septum is usually intact. </li></ul>
  16. 16. HLHS - Pathophysiology <ul><li>The major hemodynamic abnormalities are: </li></ul><ul><ul><li>Inadequate maintenance of the systemic circulation. </li></ul></ul><ul><ul><li>Pulmonary venous hypertension (if restrictive foramen ovale). </li></ul></ul><ul><ul><li>Pulmonary overcirculation (if moderate or large ASD). </li></ul></ul>
  17. 17. Anatomy
  18. 18. HLHS – Clinical manifestations <ul><li>Most infants are diagnosed in the first few hours or days of life. </li></ul><ul><li>A grayish blue color of the skin is soon apparent (denoting a mix of cyanosis and hypoperfusion). </li></ul><ul><li>If the PDA partially closes, signs of systemic hyporperfusion and shock predominate. </li></ul>
  19. 19. HLHS – Clinical manifestations <ul><li>Signs of HF usually appear within the first few days or weeks of life (dyspnea, hepatomegaly, low cardiac output). </li></ul><ul><li>Peripheral pulses may be weak or absent. </li></ul><ul><li>Cardiac enlargement is usual, with a right ventricular parasternal lift. </li></ul><ul><li>A nondescript systolic murmur is usually present. </li></ul>
  20. 20. HLHS – Clinical manifestations <ul><li>Extracardiac anomalies (particularly of the kidney and CNS, but also diaphragmatic hernia, omphalocele and hypospadia) may be present. </li></ul>
  21. 21. HLHS – Diagnostic tools <ul><li>CXR : </li></ul><ul><ul><li>Heart variable in size (1 st day of life). </li></ul></ul><ul><ul><li>Cardiomegaly develops rapidly, associated with increased pulmonary vascularity. </li></ul></ul>
  22. 22. Chest X-ray of a two-day- old boy with HLHS
  23. 23. HLHS – Diagnostic tools <ul><li>Electrocardiogram : </li></ul><ul><ul><li>Initially, may show only the normal right ventricular predominance. </li></ul></ul><ul><ul><li>Later, P waves become prominent and right ventricular hypertrophy is usual. </li></ul></ul>
  24. 24. ECG
  25. 25. ECG
  26. 26. HLHS – Diagnostic tools <ul><li>Echocardiogram : </li></ul><ul><ul><li>Is diagnostic . </li></ul></ul><ul><ul><li>There is absence or hypoplasia of the mitral valve and aortic root, a variable small left atrium and left ventricle, and a large right atrium and right ventricle. </li></ul></ul><ul><ul><li>The size of the atrial communication can be assessed directly and by pulsed and color flow Doppler studies. </li></ul></ul>
  27. 27. HLHS – Diagnostic tools <ul><li>Echocardiogram (cont.): </li></ul><ul><ul><li>Suprasternal notch views identify the small ascending aorta and transverse aortic arch (may also demonstrate a discrete CoAo). </li></ul></ul><ul><ul><li>Doppler demonstrates the absence of anterograde flow in the ascending aorta and retrograde flow via the ductus arteriosum. </li></ul></ul>
  28. 28. RA RV LA LA NORMAL 4-CHAMBER VIEW LV
  29. 29. 4-CHAMBER VIEW - HLHS
  30. 30. 4-CHAMBER VIEW – AORTIC ATRESIA WITH MITRAL ATRESIA
  31. 31. NORMAL SHORT-AXIS VIEW AO RV PA RA LA
  32. 32. SHORT-AXIS VIEW - HLHS
  33. 33. LA LV AO RV NORMAL LONG-AXIS VIEW
  34. 34. LONG-AXIS VIEW - HLHS
  35. 35. HLHS – Diagnostic tools <ul><li>Cardiac Catheterization and Angiography : </li></ul><ul><ul><li>These are no longer routinely necessary for the diagnosis of HLHS. </li></ul></ul><ul><ul><li>In patient with severe restriction at the interatrial level, a ballon septostomy would be indicated if the patient is not a candidate for surgical palliation at that time. </li></ul></ul>
  36. 36. HLHS - Treatment <ul><li>There is variable success in the surgical therapy of HLHS. </li></ul><ul><li>Management options include: </li></ul><ul><ul><li>Norwood procedure </li></ul></ul><ul><ul><li>Heart transplantation </li></ul></ul><ul><ul><li>Supportive expectant care </li></ul></ul>
  37. 37. HLHS - Treatment <ul><li>Norwood procedure: Preoperative management </li></ul><ul><ul><li>correction of acidosis and hypoglycemia </li></ul></ul><ul><ul><li>maintenance of the patency of the PDA (with PGE1) to support systemic blood flow </li></ul></ul><ul><ul><li>prevention of hypothermia </li></ul></ul><ul><ul><li>21% FiO2, with addition of 2% to 4% CO2 (PCO2 aimed at 50, pH 7.30) </li></ul></ul><ul><ul><li>Selective use of small amount of inotropic agents (sepsis or RV failure) </li></ul></ul>
  38. 38. BEFORE NORWOOD PROCEDURE
  39. 39. HLHS - Treatment <ul><li>Norwood Procedure: First Stage </li></ul><ul><ul><li>Atrial septectomy. </li></ul></ul><ul><ul><li>Transection and ligation of the main pulmonary artery. </li></ul></ul><ul><ul><li>Connection of the proximal pulmonary artery to the transversely opened hypoplastic aortic arch (“neoaorta”). The coarcted segment of the aorta is repaired. </li></ul></ul>
  40. 40. HLHS - Treatment <ul><li>Norwood Procedure: First Stage (cont.) </li></ul><ul><ul><li>Modified Blalock-Taussig shunt. </li></ul></ul><ul><ul><li>90% early survival (if in a timely fashion at selected institutions). </li></ul></ul>
  41. 41. Norwood: Stage I
  42. 42. AFTER NORWOOD STAGE I
  43. 43. HLHS - Treatment <ul><li>Norwood Procedure: Second Stage </li></ul><ul><ul><li>All prior shunts are removed. </li></ul></ul><ul><ul><li>Glenn anastomosis (bidireccional cavopulmonary procedure): the superior vena cava is connected to the pulmonary arteries. </li></ul></ul><ul><ul><li>5% mortality. </li></ul></ul><ul><ul><li>Usually performed around 6 months of age. </li></ul></ul>
  44. 44. AFTER NORWOOD STAGE II
  45. 45. HLHS - Treatment <ul><li>Norwood procedure: Third Stage </li></ul><ul><ul><li>Modified Fontan Procedure (cavopulmonary isolation): the inferior vena cava is connected to the pulmonary arteries, via either an intra-atrial of external baffle. </li></ul></ul><ul><ul><li>10-12% mortality. </li></ul></ul><ul><ul><li>Usually performed around the age of 2 years. </li></ul></ul>
  46. 46. After Stage III of Norwood Procedure
  47. 47. HLHS - Treatment <ul><li>After the third stage, all systemic venous return enters the pulmonary circulation directly. </li></ul><ul><li>Pulmonary venous flow enters the left atrium and is directed across the atrial septum to the tricuspid valve, and subsequently to the right (now the systemic) ventricle. </li></ul>
  48. 48. HLHS - Treatment <ul><li>Blood leaves the right ventricle via the neoaorta, which supplies the systemic circulation. </li></ul><ul><li>Coronary blood flow is provided by the old aortic root, now attached to the neoaorta. </li></ul>
  49. 49. HLHS - Treatment <ul><li>Cardiac Transplantation: </li></ul><ul><ul><li>It is an alternative, either </li></ul></ul><ul><ul><ul><li>In the immediate neonatal period (obviating stage I of the Norwood procedure) </li></ul></ul></ul><ul><ul><ul><li>After a successful stage I Norwood procedure </li></ul></ul></ul><ul><ul><li>Patients have the chronic risk of an organ rejection and lifelong immunosuppressive therapy. </li></ul></ul>
  50. 50. HLHS – Treatment <ul><li>Five year survival: </li></ul><ul><ul><li>70% for staged palliation (Norwood procedure). </li></ul></ul><ul><ul><li>75% for transplantation. </li></ul></ul>
  51. 51. HLHS - Treatment <ul><li>Supportive expectant care (“do nothing”) </li></ul><ul><ul><li>Especially true when multiple noncardiac congenital anomalies exist or when serious multiorgan system damage is present. </li></ul></ul>
  52. 52. HLHS – Pitfalls for the Nursery <ul><li>Avoid using oxygen despite low pulse oximetry saturation. </li></ul><ul><li>Increasing FiO2 will lower pulmonary vascular resistance and increase blood flow to the lungs, which are already “overcirculated”, thereby worsening systemic perfusion. </li></ul>
  53. 53. HLHS – Pitfalls for the Nursery <ul><li>Avoid overventilating the infant. </li></ul><ul><li>Carbon dioxide is a pulmonary vasoconstrictor and may improve systemic perfusion and cardiac output. Try to maintain normal or mildly elevated PaCO2 levels. </li></ul>
  54. 54. HLHS - Summary <ul><li>It is a collective term describing a group of cardiac malformations that share various degrees of hypoplasia of the structures of the left side of the heart. </li></ul><ul><li>Over 95% of patients with HLHS will die if left untreated during the first month of life. </li></ul><ul><li>Echocardiogram is the diagnostic procedure. </li></ul><ul><li>Surgical intervention has become a medical standard. </li></ul>

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