Nine days old male was admitted to the intensive care unit because of the jaundice and suspicious cardiac disease. He was delivered at 39 +5 weeks of gestation without peripartum events. His birth weight was 3.4 kg. He was the second baby with healthy brother. The family history was not contributory.
He was initially transferred to the other hospital because of the jaundice at 2 days of age. Cardiac murmur was detected at that hospital. He was asymptomatic besides mild dyspnea. He was able to suck the milk of 70 ml per feeding.
He was referred to another hospital because the echocardiographic examination showed a small left ventricular size

Chi, Je G.

Seo, Jeong Wook

Sohn, Sejung

SNU Open Repository and Archive

The Seoul Journal of Medicine Vol. 36, No. 2197-103, June 1995 Hypoplastic Left Heart Syndrome with Ventricular Septal Defect and Patent Arterial Duct (SNUCH CPC 95-3) Moderator : Je G. Chi1 Discussant : Sejung Sohn2 Pathologist : Jeong Wook Seo1 Department of Patlzology , Seoul National Universitj, College of Medicine, Seoul, Korea, Department of  Pediatrics2, Sejong General Hospital, Buchon-si, Korea CASE HISTORY Nine days old male was admitted to the in- tensive care unit because of the jaundice and suspicious cardiac disease. He was delivered at 3gf5 weeks of gestation without peripartum events. His birth weight was 3.4kg. He was the second baby with healthy brother. The family history was not contributory. He was initially transferred to the other hospi- tal because of the jaundice at 2 days of age. Cardiac murmur was detected at that hospital. He was asymptomatic besides mild dyspnea. He was able to suck the milk of 70ml per feeding. He was referred to another hospital because the echocardiographic examination showed a smal! left ventricular size. Physical examination at the emergency room revealed a mildly lethargic baby with dyspnea. Body weight was 3.28 kg and height 52cm. His body temperalure was 36.2Y2, heart rate 1501 min, respiration rate 761min. The blood pressure on the right arm was 58/33mmHg, left arm 641 37mmHg, right leg 64136mmHg and left leg 58/36 mmHg. He was slightly anemic and icteric. Held on April 1 1. 1995 at 1 :00 p.m. in the auditorium I1 of the Seoul National University Children's Hospital. A j $ q q i i L  q q q q  tgqqiiLg : q4]3, ~ j Z d + -  ~-1~gtgq &0\4 : $A]] ~2 There was no definite cyanosis. Venous engore- ment was not present. Pulse volume on the extremities seemed slightly decreased. Chest examination showed symmetric expansion of  the chest with moderate retraction. The breathing sound was clear without rales. Cardiac examin- ation revealed right ventricular impulse, single S2 at left upper sternal border, and grade 216 sys- tolic murmur along the .left sternal border. Dias- tolic murmur was not clearly heard. His abdo- men was slightly protruded. The liver was palpat ed four fingerbreaths below subcostal margin. The spleen was impalpable. There was no de- formity on the extremities. The blood gas analysis at the emergency room was pH 7.39, PaC02 35mmHg, Pa02 62mmHg and bicarbonate 21 mmol1L. CBC was Hb 10.8 gm/dl, Hct 31.4%, WBC 5.7X 1 03/mm3 and RBC count 3.1 9 X  1 06/mm3. Electrolytes were Na 130 mmol1L. K5.3mmol/L and CI 101 mmol/L, and tot- al CO2 18 mmol/L. The other chemistry results were, BUN, 4mg/dl creatinine, 0.2 mg/dl ; bilirubin 9.5 gm/dl, glucose, 132 mgldl and ionized calcium, 1.04mmol/L. The urinalysis was normal. The infan- togram revealed increased pulmonary vascularity. The echocardiographic examination showed nor- mal connections, small left ventricle, large right atrium, large right ventricle and enlarged pulmon- ary artery. After admission to the neonatal intensive care- unit, intravenous PGEt (0.01 pgmIKglmin), dob- utamine(5pgm/Kg/m1n), digoxin(16.4pgm every 12 hours) and lasix(2 mg every 12 hours) were star- ted. Ampicillin and gentamicin were also admin- istered for the possibility of sepsis. About three hours after intravenous treatment, his respiration became irregular and intermittently apneic. Oxy- gen saturation was around 85%. There was no self voiding since admission. Because of the res- piratory difficulty, oxygen administration at the flow rate of 5 Llmin by means of hood was star- ted. Additional intravenous diuretics were tried wit- hout effect. Oxygen saturation was monitored above 90% at that time. Blood gas analysis show- ed increased acidosis(7.30-40-79-19). Because of persistent oliguria and acidosis, ven- tilator therapy was started as be ;  FiO2 0.3, venti- lator rate 20/min, PIP 20cmH2, PEEP 3cmH2, mean airway pressure 3 c m H ~ ;  I :  E rate 1 : 7.4, in- spiratory time 0.35 sec, and flow rate 10LImin. Urine output was not responsive to normal saline volume challenge. In the mean time, there was moderate amount of bronchial secretion and Sa02 fluctuated markedly on stimulation. Con- trolled ventilator settings and respiratory paralysis were applied because of tachypnea(50-60/min), metabolic acidosis(ABGA 7.33-26-71 -1 3- 6.95-1 5 -49-3) and hyperkalemia (7.2 mmol1L). New setting were Fi02 0.21, rate 16lmin ( -  l3/min), PIP 20 cmH2, I : E ratio 1 : 9.5, inspiratory time 0.44 sec. Blood gas data after new ventilator setting was 7.02-59-36-14. Thereafter, pulse volume became increased and urine output became responsive to diuretics. SaOz was maintained around 70%. The follow-up blood gas was 7.26-57-31 -25. His parents rejected medical advice for oper- ation and took the baby home on the 3rd hospi- tal day (1 1 days after birth). he died soon after his discharge. DISCUSSION Dr. Sohn. Some clinical history is to be added to the protocol before we discuss this case. Was there any urine output when he was in the emer- ge'ncy room? How about his temperature at that time? Was the proportion of direct and indirect bil- irubin available? Was this patient treated for anemia? Dr. Chung II Noh(Pediatrics, Seoul National University Children's Hospital). I was the pedia- trician in charge of this baby. We were informed that he voided before his arrival at the emergency room, but not in our hospital. He was normother- mic but rather cold. Direct and total bilirubin were 0.619.5 gmldl. We transfused packed cells, and hemoglobin level after transfusion was 13.3 gmldl and hematocrit was 42.6 O/O. Dr. Sohn. This baby had a jaundice from the second day, and small left ventricle was detected by echocardiography which was performed be- cause of cardiac murmur. This tachypneic baby was not severely cyanotic. Arterial pulse was slig- htly decreased at the extremities without blood pressure difference between upper and lower extremities. Positive cardiac and hemodynamic fin- dings on physical examination were increased rig- ht ventricular impulse, single S2 sound, systolic murmur and hepatomegaly. Laboratory study rev- ealed anemia, leukopenia, and hyperbilirubinem- ia. Arterial gas analysis data showed a significant change by the treatment at the neonatal intensive care unit. Initial data at the emergency room was still good, but after treatment including oxygen supply, metabolic acidosis became apparent while arterial oxygen saturation was still good. Persist- ent acidosis and oliguria were major problems. The ventilator setting was changed to maintain respiratory acidosis. Low oxygen fraction of the inspired gas(Fi02 0.21 ) and hypoventilation (res- piratory rate decreased from 16 to 13) could al- leviate the metabolic acidosis, and arterial pulse of the extremities became stronger. Urinat~on was noted at that time. Dr. In-One Kim (Radiology). This baby had a chest radiogram, which revealed cardiomegaly and increased pulmonary vacularity. However, pul- monary venous congestion was not conspicuous. Dr. Sohn. In summary. This nine days old male had a heart failure, increased pulmonary vascu- larity and a small left ventricle on echocardiograp hy. Metabolic circuit in this patient could be sum- marized as in figure 1 (Lirenman 1993). Metabolic acidosis with moderate arterial oxygenatioii sug- gests cardiac lesions with low cardlac output and increased pulmonary blood flow. There remains, however, a possibility of sepsis. Differential d ~ a g -  nosis of heart failure in a neonate could be listed -99 - Table 1. Causes of cardiac failure by the age of onset in a newborn baby. 1 .  At b~ r th  : 1) hydrops fetalis associated with intrauterine hemolysis, arrhythmia or myocarditis 2. The first day 1 ) volume overload : tricuspid regurgitation (Ebstein's anomaly), pulmonry valve regurgitation 2) arteriovenous fistula 3) hypoplasia of the left heart 4) total anomalous pulmonary venous return with obstruction 5) myocardial ischemia due to birth asphyxia 6) metabolic causes, e.g. hypoglycemia, anemia, hypocalcemia. 7) paroxysmal tachycardia 3. The second day to one week 1 )  severe left ventricular outflow tract obstruction : hypoplastic left heart syndrome, interruption of the aortic arch, severe coarctation of aorta, critical aortic stenosis. 2) complete transposition of the great arteries 3) Patent arterial duct in premature baby 4) critical pulmonary valve stenosis ~~ - --- as in table 1. Absence of edema excludes a possibility of fet- al hydrops and tricuspid valvular lesions. Small left ventricle excludes a possibility of primary myocardial disease, arteriovenous fistula and other metabolic causes of heart failure. Most pos- s~ble conditions in t h ~ s  patient, therefore, are a syndrome with a left ventricular hypoplasia and anomalous pulmonary venous return with pul- monary venous obstruction or varieties of univ- entricular heart without pulmonary stenosis in- cluding atrial iscmerism complex. It is rather diffi- cult to point out the cardiac condit~on without echocard~ography. Univentricular heart without pulmonary stenos~s, however, usually accompany congestive failure around 2 weeks of age due to decrease of the vascular resistance of the pul- monary bed. Total anomalous pulmonary venous return with venous obstruction may have a simi- lar cl~nical features to hypoplastic left heart syn- drome. Absence of significant hypoxemia and cy- anosls, and radiologic findings denying pulmon- ary venous congestion suggest that total anomal- ous pulmonary venous return is less likely. Hypop- lastic left heart syndrome is the most likely con- d~t ion.  Aortic and mitral valves are stenotic or atretic in a heart with a hypoplastic left ventricle. Most common form is atresia of both mitral and aortic valves (Castaneda et a/., 1994, Backer et a/., 1994). Hearts with ventricular septal defect are rare (5%) but they have a left ventricle with a rather good size, and biventricular repair could be an option for surgery (Austin et a/ . ,  1989). Hearts without ventricular septal defect have a diminutive left ven- tricle and biventricular repair is not possible (Bac- ker et a/ . ,  1994). After birth of a baby with a hypoplastic left heart syndrome, there are two sig- nificant hemodynamic alterations. Narrowing or closure of arterial duct markedly decrease sys- temic arterial perfusion including coronary arterial blood flow. Ratio of pulmonary and systemic vas- cular resistance (RpIRs) can determine the clinical features. Decrease of the pulmonary vascular re- sistance and increase of the systemic arterial re- sistance make the lung plethoric and systemic bed oligemic(Norwood 1991). The clinical feat- ures o f  hypoplastic left heart syndrome, therefore, depends significantly on the condition of the inter- atrial communication, whether it is restrictive or not (Table 2) (Castaneda et a/ . ,  1994, Sohn et a/ . ,  1986). acidosis hvfotension perfusion Fig. 1. Metabolic circuit responsible for the heart failure in this patient. Dr. Jung Yun Choi (Pediatrics, Seoul National University). Which do you think is more import- ant for this patient as a cause of death, ductal closure or decrease in pulmonary venous resist- ance? Dr. Sohn. Sudden closure of the arterlal duct could be a direct cause of death. Decrease of pulmonary venous resistance, however, would be more important because I don't think ductus is significantly narrowed in this patient. Dr. Noh. In the management of this patient it is important to recognized that baby with hypop- lastic left heart syndrome should not be main- tained in a state of a high oxygen tension. This disease is often claimed by the parents that the baby became worse after the medical care at the hospital. The principle of med~cal care in t h ~ s  patient is to balance the pulmonary and systemic resistance In order to keep adequate blood flow and adequate oxygen content. Low oxygen tension and hlgh carbon dioxide tension in the Inspired gas would control pulmonary blood flow by increasing pulmonary vascular re- slstance and prevent circulatory collapse. Non- restrictive atrial septal defect decreases pulmon- ary vascular resistance without pulmonary ve- nous hypertension. Some degree of pumonary venous hypertension IS necessary in early neo- natal period to malntain the systemic arterial pressure through the arterial duct and to de- crease the pulmonary arterlal blood flow. This nonrestrictive atrial septal defect is one of caus- es of the low cardlac output and moderate de- gree of arterial oxygen saturation. Cltnical Diagnosis (Dr. Sohn) 1.  Hypoplastic left heart syndrome 2. Mildly restrictive lnteratrial communlcatlon PATHOLOGICAL  FINDINGS (Dr. Jeong-Wook Seo ; Patholgy) Echocardiography has a crucial role in the clinical management in thls patlent. Echocardlog- ram performed by Drs. Chung I1 Noh and Ho Sung Kim (Seoul Natlonal University Hospital) Table 2. Spectrum of clinical profiles by the amount of interatrial commun~cation in hypoplastic left heart syndrome. Groups I I I I I I Interatrial restrictive unrestrict lve communication (low pulmonary resistance) QpIQs -- 1 .o >> 1 .o Major apnea systemic complications hypoperf usion Management plan ventilation on apnea correct~on of  acidosis hypoventllatlon high C02 in the lnsplred gas excessively restrict lve (high pulmonary resistance) << 1 .o profound hypoxemia hyperventilation high Fi0z increase system~c vascular resistance hypot hermia lmmedlate surgery showed small left ventricle, large ventricular sep- tal defect of muscular type, nonrestr~ctive atrlal septal defect, mitral atresia and largely patent arterial duct. Coarctation of aortic isthmus was suspected. These findings reflect the autopsy fin- dings very well. Autopsy revealed congenital cardiac lesions of hypoplastic left heart syndrome. Mild cardiom- egaly was present and small left ventricle was hidden on the left lateral aspect of a large right ventricle in the frontal aspect of the heart (Fig. 2). A redundant flap of oval fossa with an open- ing, a muscular ventricular septal defect were seen from the right ventricular aspect(Fig. 3) and the left ventricular aspect (Fig. 4). Figure 4 also shows that the left ventricle was not very much hypoplastic. Mitral valve was completely atretic. There was no valvar apparatus. Bicuspid stenotic aortic valve was present. Coarctation of  the aorta and largely patent arterial duct were seen (Fig. 2, 5). Histologic study of lungs show- ed congestion of pulmonary veins but interstit- ium was not edematous. Pulmonary arterles didn't show hypertensive change at all. There was no ev~dence of sepsis or disseminaed intrav ascular coagulation. Liver was enlarged but his- tologic findlngs were within normal limit. Kidneys showed patchy hemorrhage, suggestive of ischemic change in this organ. The degree of the left ventricular hypoplasia was not clearly mentioned in the protocol but it was not such small as in a case with atretic aortic and mitral valves. Presence of the ven- tricular septal defect and coarctation of aorta explains why the left ventricle was not so big as in a case with hypoplastic left heart syndrome. Even though there was coarctation of the aorta in a moderate degree, large arterial duct, large ventricular septal defect, relatively good ascend- ing aorta explain the absence of discrepancy in the arterial pressure at extremities. Pulmonary artery was slightly small but decrease in the pul monary vascular resistance resulted in the increased demand to the ventricular output, and Fig. 2. Frontal aspect of the autopsied heart showing coarctation of aorta (open ar- row) and patent arterial duct (DA). As- cending aorta (AO) is only mildly hypop- lastic compared to the pulmonary trunk (PT). Right ventricle occupied the whol- d anterior wall of the heart, whereas only a thin rim of the left ventricle(LV) is seen from this frontal aspect. Right atrium (RA) is enlarged. Fig. 3. Rlght atrlal and ventrlcular aspect of the autopsted heart showlng muscular sl~t-like defect (open arrow) at the rlght ventrlcular trabecular zone Atrlal sep- tum IS redundant and patent oval for- amen (PFO) IS open as an lnteratrtal communlcatton Closed arrow lndlcates pulmonary arter~al outlet (IVC Inferlor caval ve~n)  Fig. 4. Left ventricular aspect shows mild hy- poplasia of the ventricular volume, mi- tral atresia (Black arrows) and bicuspld stenotlc aortic valve (AO). Ventricular septal defect (white arrow) is also seen. f~nally resulted In the heart fallure. It I S  not certain why this baby had anemla, hyperblllrublnemia and leukopenia. I suppose general circulatory instability was superimposed to the physlologlc jaundice and subcl~nlcal sep- SIS. Pathologic diagnosis 1 .  Hypoplastic left heart syndrome : 1 ) Mitral atresla 2) Small left ventricle 3) Aortlc stenosls and blcuspid valve 4) Ventr~cular septal defect, muscular 5) Patent oval foramen 6) Coarctation of aorta, isthmus 7) Patent arterial duct 2. Congestwe hepatomegaly 3. Pulmonary congestion 4, lschemlc hemorrhage, kldneys Fig. 5. Opening the pulmonary trunk and arter. ies show two openings for the right and left pulmonary arteries (open arrow- s). Blood flow to the descending aorta (D-AO) is from the right ventricle(RV) through the pulmonary valve and the arterial duct (DA), which has a wrinkled intimal surface. A small opening distal to the arterial duct (black arrow) is the aortic arch with coarctation, which is connected to the ascending aorta (A-AO). REFERENCES Aust~n EH, Jonas RA, Mayer JE, Castaneda AR. Aortic atresia wlth normal left ventricle. Single-stage repair in t heneonate. J Thorac Cardlovasc Surg 1989 ; 97 : 392-5 Backer CL, Bove EL, Zales VR, Mavroudis C. Hypoplastic left heart syndrome. In Mavroudis C, Backer CL (Eds) Pediatric cardiac surgery. 2nd ed. Mosby-Year Book Inc. St. Louis, 1994, pp. 442-53 Castaneda AR, Jonas RA, Mayer JE, Hanley FL. Cardiac surgery of the neonate and infant. WB Saunders Co, Philadelphia 1994, pp. 363- -85 Lirenman DS. Flulds and electrolytes. In LeBlanc JG, Willlams WG (Eds) The operative and pos- toperatlve management of  congenital heart defects. Futura Publishing Co, Mount Kisco, 1993, pp. 21 7-41 Norwood WI. Hypoplastic left heart syndrome. Ann Thorac Surg 1991 ;52:688-95 Sohn SJ, Koh JG, Choi JY, Yoon YS, Hong CY, Kwon TJ. Hypoplastic left heart syndrome -2 cases report-. Kor Circulation J 1986 ; 16 : 401 -9 

Hypoplastic Left Heart Syndrome with Ventricular Septal Defect and Patent Arterial Duct (SNUCH CPC 95-3)

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Hypoplastic Left Heart Syndrome with Ventricular Septal Defect and Patent Arterial Duct (SNUCH CPC 95-3)

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