Resuscitation of a Newborn with Right Hypoplastic Heart Syndrome: A Rare Confrontation for an Anesthesiologist.

According to newborn resuscitation guidelines, all referrals for neonates with suspected or confirmed duct-dependent congenital heart disease are to be discussed with pediatric cardiologist beforehand and are to be transferred immediately under their care for optimal management. However, in case of emergency, when there is not adequate time for preoperative consultations or a multidisciplinary approach, we should be able to manage these patients in the immediate perioperative period to decrease the likelihood of adverse outcome. We herewith describe a case where we as anesthesiologists successfully resuscitated a newborn with right hypoplastic heart in an emergency case of cesarean section till the baby was transferred to level III cardiac institution for further management.

INTRODUCTION

Congenital heart diseases (CHDs) account for 30%–50% of infant deaths.[1] CHD covers a wide spectrum of defects where there is either obstruction of flow to the blood in the heart or the blood takes an abnormal route through the heart, and very rarely, the right/left side of the heart has failed to form properly (hypoplastic heart). We present our case where our patient had right hypoplastic heart.

CASE REPORT

A 26-year-old female with a history of primary infertility for 6 years conceived by in vitro fertilization at our institute. An antenatal ultrasonography (USG) at 20 weeks of gestation showed a fetus with right hypoplastic heart. This was further confirmed by fetal two-dimensional echocardiography which showed that the right atrioventricular (AV) valve was atretic with no antegrade flow through it. Although there were four chambers in the heart, the right ventricle (RV) was very small. Hypoplastic pulmonary artery, atrial septal defect (ASD), and moderate ventricular septal defect (VSD) were also seen. The case was discussed with a cardiology consultant who assured that a pediatric cardiologist would be available at our institute for newborn resuscitation at the time of elective cesarean section. In case of delivery during emergency hours, if the baby was stable, the cardiologist was to be consulted during routine hours.

At 36 weeks of gestation, the mother had leaking per vaginum and was immediately taken up for emergency cesarean section. A call was sent to the pediatric cardiologist. We kept everything ready for neonatal resuscitation - armamentarium for endotracheal intubation and ventilatory support, cardiac drugs for resuscitation, inotropes and vasopressors with infusion pumps, and prostaglandin E1 (PGE1) infusion.

The mother was given spinal anesthesia in L3–L4 interspace using Quincke's needle, and 2.2cc bupivacaine heavy was injected to achieve T6 sensory level. The patient was hemodynamically stable throughout the surgery. A female child weighing 2.1 kg was delivered. Apgar score was 8 at 1 and 5 min. Baby's heart rate was 140/min, and no murmurs were appreciated. Peripheral pulses were good. Respiratory rate was 40/min, with clear lungs and no distress. She had central cyanosis. Her saturation on room air in the right hand was 81%. We did not give supplemental O2. A 24-gauge intravenous line was inserted. We observed her for some time and then shifted her to the cardiac institute for further management.

DISCUSSION

In right hypoplastic heart syndrome, the tricuspid valve is absent, and the RV is hypoplastic in absence of the inflow portion of the RV.[2] In majority of such cases, pulmonary stenosis is present with resulting hypoplasia of pulmonary arteries and reduced pulmonary blood flow (as in our case). The heart is unable to properly oxygenate the blood. Because of the lack of an AV connection, an ASD must be present to maintain blood flow. Furthermore, since there is a lack of RV, there must be a way to pump blood into pulmonary arteries, and this is accomplished by a VSD. Because the only way, the pulmonary circulation receives blood is through the VSD, and a patent ductus arteriosus usually also persists to increase pulmonary blood flow.

Infants present with cyanosis which is usually severe from birth, tachypnea, and poor feeding. PG is started in neonates with severe cyanosis to maintain ductal patency before planned cardiac surgery. Few survive beyond 6 months of age without surgical intervention.

Our main aim during resuscitation was to keep the ductus arteriosus patent and increase the pulmonary blood flow.[3] After birth, the baby was wiped thoroughly and warmed properly. The extremities and tongue showed cyanosis. On room air, her SpO2 was 81% with good respiratory effort. We did not give supplemental O2 because the primary stimulus for the closure of ductus arteriosus is increase in neonatal blood O2. O2 is to be given in these cases when SpO2 is <75% with PGE1. We also secured an intravenous line, and in case, there was a need to give PG infusion. PGE1 has vasodilator properties and prevents the premature closure of ductus arteriosus.[4] Some of the side effects of PG are hypotension, hypoglycemia, fever, and apnea. Keeping this in mind, we monitored the pulsations in all the four limbs, kept vasopressors and inotropes ready to maintain adequate systemic perfusion (and encourage pulmonary perfusion by increasing systemic vascular resistance) in case hypotension occurred. Ventilatory support was also kept ready should apnea occur. When we were sure that the baby was maintaining SpO2 of 82% on air with good respiratory effort and she did not require PGE1 infusion in the immediate postdelivery period, the baby was shifted to the cardiac institute for further management with ready availability of ventilatory support in the transport setting.

CONCLUSION

With modern day intensive neonatal care and multidisciplinary approach, the chances of survival of newborns with major CHD have increased significantly. However, in case of emergencies where a Neonatal Intensive Care Unit setup is not available, the anesthesiologists should be familiar with the basics of neonatal resuscitation in such high-risk cases for safe and effective perioperative care of the patient to decrease the likelihood of adverse outcome.

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Conflicts of interest

There are no conflicts of interest.