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December 2011 Hypoplastic Left Heart Syndrome in in THIS ISSUE the Neonate by Srilatha Alapati, MD and P

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December 2011 Hypoplastic Left Heart Syndrome in in THIS ISSUE the Neonate by Srilatha Alapati, MD and P NEONATOLOGY TODAY News and Information for BC/BE Neonatologists and Perinatologists Volume 6 / Issue 12 December 2011 Hypoplastic Left Heart Syndrome in IN THIS ISSUE the Neonate By Srilatha Alapati, MD and P. Syamasundar other complex forms of congenital heart disease Hypoplastic Left Heart Syndrome in whom a two-ventricle repair is not possible. in the Neonate Rao, MD by Srilatha Alapati, MD and P. Syamasundar Rao, MD In this review, we will discuss anatomic, physiol- Page 1 Introduction ogic, and clinical features, noninvasive and inva- sive evaluation, management, and prognosis of A Brief History of Xenon and In the past, we discussed several general topics HLHS. Neuroprotection about Congenital Heart Disease (CHD) in the by Meredith Holmes, BA and Jerold F. neonate,1-5 but recently we began addressing Pathological Anatomy Lucey, MD, FAAP individual cardiac lesions such as transposition of Page 10 the great arteries6 and Tetralogy of Fallot.7 In this The left ventricle is usually a thick-walled, slit-like issue of Neonatology Today we will discuss Hy- cavity, especially when mitral atresia is present. DEPARTMENTS poplastic Left Heart Syndrome. Usually the aortic valve is severely stenosed or Medical Meeting Focus atretic and its annulus hypoplastic. Similarly, the Page 9 Hypoplastic Left Heart Syndrome mitral valve may be hypoplastic, severely stenotic or atretic. When the mitral valve is open, the left Medical News, Products and The term, Hypoplastic Left Heart Syndrome ventricular cavity is very small. Endocardial fi- Information (HLHS), initially proposed by Noonan and broelastosis is usually present. The ascending Page 11 Nadas,8 describes a diminutive left ventricle with aorta is hypoplastic; its diameter may be 2 to 3 underdevelopment of mitral and aortic valves. A mm or less. While it is very small, it is sufficient to NEONATOLOGY TODAY patent foramen ovale or an atrial septal defect is supply adequate coronary blood flow in a retro- Editorial and Subscription Offices usually present. The ventricular septum is usually grade fashion. The left atrium is also small, re- 16 Cove Rd, Ste. 200 intact. A large patent ductus arteriosus supplies flecting the limited blood flow in utero. The atrial Westerly, RI 02891 USA www.NeonatologyToday.net blood to the systemic circulation. Coarctation of septum is usually thickened; the foramen ovale the aorta is also commonly present. may be small and, occasionally, it may be closed. Neonatology Today (NT) is a monthly A patent ductus arteriosus is usually present and newsletter for Neonatologists and Peri- Hypoplastic Left Heart Syndrome is a uniformly is required for survival. natologists that provides timely news and information regarding the care of lethal cardiac abnormality unless it is surgically newborns and the diagnosis and treat- addressed. Since the description of surgical pal- A severely hypoplastic left ventricle may also be ment of premature and/or sick infants. liation by Norwood in the early 1980s9,10 and the present in hearts with double-outlet right ventricle report of allograft cardiac transplantation by Bai- with mitral atresia, unbalanced complete atrio- © 2011 by Neonatology Today ISSN: 11 1932-7129 (print); 1932-7137 (online). ley in the mid 1980s for treatment of HLHS, the ventricular canal and other complex heart de- Published monthly. All rights reserved. interest in this lesion has remarkably increased. fects; in some studies, these variants constitute The Norwood surgical approach consists of a as many as 25% of Hypoplastic Left Heart Syn- Statements or opinions expressed in Neo- series of three operations: Norwood procedure dromes patients.12 natology Today reflect the views of the authors and sponsors, and are not neces- (Stage I), hemi-Fontan or bidirectional Glenn sarily the views of Neonatology Today. procedure (Stage II), and Fontan conversion Pathophysiology (Stage III). Orthotopic heart transplantation pro- vides an alternative therapy, with results similar to Prenatal Circulation Upcoming Medical Meetings those of the staged surgical palliation. Currently, (See website for additional meetings) the survival rate of infants treated with these sur- In utero, the oxygenated blood from the pla- NeoPREP: An Intensive Review gical approaches is similar to that of infants with centa is returned to the inferior vena cava, and Update of Neonatal- Perinatal Medicine Jan. 21-27, 2012; New Orleans, LA USA NEONATOLOGY TODAY eweb.aap.org/NeoPREP CALL FOR PAPERS, CASE STUDIES NEO: The Conference for AND RESEARCH RESULT Neonatology Feb. 23-26, 2012; Orlando, FL USA www.neoconference.com Do you have interesting research results, observations, human in- SR2.0: Speciallty Review in terest stories, Neontology and Perinatology 2.0 reports of meetings, etc. to share? Feb. 23-27, 2012; Orlando, FL USA www.specialityreview.com Submit your manuscript to: [email protected] Recruitment Ads: Pages 2 and 8 which instead of shunting across the patent saturation, systemic blood flow is decreased. was responsible for 25% of cardiac deaths in foramen ovale into the left atrium mixes with When the systemic blood flow decreases be- the neonatal period.15 There is a higher inci- the superior vena caval blood in the right low a critical level, the perfusion becomes poor, dence of HLHS in patients with Turner Syn- atrium. The pulmonary venous blood return- and metabolic acidosis and oliguria may de- drome, Noonan Syndrome, Smith-Lemli-Opitz ing into the left atrium is shunted across the velop. There is also decreased flow to the Syndrome, and Holt-Oram Syndrome than in atrial septum because of mitral valve obstruc- coronary arteries and brain, with a risk of myo- normal children. Certain chromosomal duplica- tion. This mixed right atrial blood then crosses cardial or cerebral ischemia respectively. Alter- tions, translocations, and deletions are also the tricuspid valve into the right ventricle and natively, if pulmonary vascular resistance is associated with HLHS. into the pulmonary artery.13 Complete admix- significantly higher than systemic vascular ture of all venous return (that from the pla- resistance, there will be hypoxemia. Gender centa as well as the fetus) results in blood flow with identical oxygen saturation to all parts of In summary, the postnatal circulation in HLHS The HLHS is more common in males than in the fetal heart. Because of widely patent duc- depends on three major factors: females, with a 55-70% male preponderance. tus arteriosus and high pulmonary vascular • Adequacy of inter-atrial communication resistance in the fetus, most of the blood is • Patency of the ductus arteriosus Age directed into the aorta via the ductus with only • Level of pulmonary vascular resistance a small portion of the blood from the main pul- Babies with HLHS typically present within the monary artery entering the branch pulmonary Epidemiology first 24-48 hours of life. Presentation occurs arteries and lungs. Once in the aorta, the blood as soon as the ductus arteriosus begins to gets distributed into the aortic arch, brachio- Prevalence constrict, thereby decreasing systemic blood cephalic vessels and ascending aorta on the flow, producing shock, and, without interven- one hand and descending aorta on the other. Recently reported prevalence of HLHS varies tion, causing death. Infants with pulmonary The quantitative distribution into these different between 0.21 and 0.28 per 1000 live births.14 It venous obstruction (absent or restrictive pat- vascular beds depends on their relative vascu- comprises 1.2-1.5% of all congenital heart ent foramen ovale) may present even sooner. lar resistances. The aortic arch and ascending defects.15 Hypoplastic Left Heart Syndrome Very rarely, an infant with persistence of high aortic blood flows in a reverse direction and accounts for 7-9% of all congenital heart dis- pulmonary vascular resistance and widely supplies the coronary arteries. ease diagnosed in the first year of life.14,15 Be- open ductus arteriosus may present later be- fore surgical treatment was available, HLHS cause of balanced pulmonary and systemic Postnatal circulation circulations. The newborn infant with HLHS has a complex Mortality/Morbidity cardiovascular physiology. Fully saturated pulmonary venous blood returning to the left Without surgery, HLHS is uniformly fatal usu- atrium cannot flow into the left ventricle be- ally within the first 2 weeks of life. As alluded to cause of atresia, hypoplasia, or stenosis of the above, survival for a longer period occurs mitral valve. Therefore, pulmonary venous rarely and is related to persistence of the duc- blood must cross the atrial septum (Figure 1). tus arteriosus along with balanced systemic This blood mixes with desaturated systemic and pulmonary circulations. venous blood in the right atrium and from there is transmitted into the right ventricle and the Following the Norwood procedure,16 overall pulmonary artery. The right ventricle then must success (survival to hospital discharge) is ap- pump this mixed blood to both the pulmonary proximately 75%. Success rates are higher and the systemic circulations that are con- (85%) in patients with no or a low number of nected in parallel, rather than in series, by the preoperative risk factors and lower (45%) in ductus arteriosus. Blood exiting the right ven- patients with important and/or multiple risk tricle may flow into the lungs via the branch Figure 1. Box diagram of Hypoplastic Left factors. The risk factors for poor result include pulmonary arteries or into the aorta via the Heart Syndrome. The pulmonary venous re- prematurity and major non-cardiac malforma- ductus arteriosus (Figure 1). The blood flow turn cannot exit into the left ventricle and its tions. Other identified risk factors include sur- into the aortic arch and ascending aorta is in a egress has to be into the right atrium via the gery in older infants, significant tricuspid regur- retrograde direction (Figure 1). patent foramen ovale (PFO). Because there is gitation, and pulmonary venous hypertension. no forward flow from the left heart into the hy- High Aristotle Scores are also associated with The relative flows to the pulmonary and sys- poplastic aorta, the systemic perfusion is de- poor prognosis.
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