Failure of the normal transition from in utero to ex utero physiology leads to “persistent” pulmonary hypertension of the newborn (PPHN). PPHN is frequently associated with low systemic blood pressure and low cardiac output because of increased right ventricular afterload and myocardial dysfunction. The general management of newborns with PPHN is geared toward maintenance of normothermia, normal serum electrolytes, normal intravascular volume, correction of acidosis, adequate sedation/analgesia, adequate ventilation and oxygenation with optimal lung recruitment, and avoidance of hyperoxia. Inotropic and vasoactive agents are commonly initiated early to increase cardiac output, maintain adequate systemic blood pressure, and enhance oxygen delivery to the tissue. Unfortunately, there is not much evidence on the choice, timing of initiation, dosing, monitoring, and titrating of vasoactive agents in this patient population. In this review, we will discuss the pathophysiology of PPHN and review the use of inotropic, lusitropic, and vasoactive agents in the management of PPHN, with particular attention to milrinone.

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Objective  This article studies the role of sildenafil in reducing myocardial stress (measured by serial N-terminal pro b-type natriuretic peptide [NTproBNP] levels) secondary to bronchopulmonary dysplasia-associated pulmonary hypertension (BPD-PH). Study Design  This is a case series of three extremely low birth weight infants with severe BPD at 36 weeks' postmenstrual age. All infants had very elevated NTproBNP (> 2,000 ng/dL) levels and echocardiographic evidence of BPD-PH. Sildenafil was started and infants were followed up every 2 weeks clinically along with NTproBNP levels and echocardiograms. Results  After 4 weeks of sildenafil treatment, NTproBNP levels decreased significantly in all infants, echocardiograms showed significant improvement in one infant, and respiratory severity score improved significantly in one infant. All infants tolerated sildenafil. Conclusion  Sildenafil reduced NTproBNP levels in all infants with BPD-PH but the echocardiographic findings and respiratory scores did not improve consistently. We speculate that this may be due to a delay in diagnosis and initiation of therapy after irreversible pulmonary changes have set in.

Objective  The use of antenatal magnesium sulfate (MgSO 4 ) has been associated with neuroprotective effects. One of its' proposed mechanisms of action includes antagonism of calcium channels. Calcium influx is important for closure of ductus arteriosus. We hypothesized that antenatal MgSO 4 exposure may be associated with an increased risk of hemodynamically significant patent ductus arteriosus (HsPDA) in premature infants (PI). Study Design  A prospective cohort study conducted in two parts. PI (< 32 weeks and < 1,500 g) were recruited ( n  = 105). All infants had Echocardiograph (ECHO; within 3 days) and blood samples drawn at the same time for B-type natriuretic peptide (BNP; part 1) and NTproBNP (N-terminal pro BNP; part 2) measurements. HsPDA was defined as a PDA diameter > 1.5 mm and BNP levels > 40 pg/mL or NTproBNP > 10,200 pg/mL. Infants were divided into two groups based on antenatal MgSO 4 exposure. Data were analyzed using SPSS 23. Difference in baseline characteristics and antenatal steroid use in the two groups was analyzed. A matched group analysis was performed to adjust for the difference in the numbers between the two groups. A p -value < 0.05 was considered significant. Results  There was no significant difference seen in baseline characteristics or use of antenatal steroids in exposed versus unexposed ( n  = 95 vs. n  = 10). There was a significant negative correlation between antenatal MgSO 4 exposure and HsPDA in PI ( p ≤ 0.05). However, this association was not significant after matched group analysis. Conclusion  Antenatal MgSO 4 exposure is not associated with an increased risk of HsPDA. It may be associated with a decreased likelihood of HsPDA.

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Background: Postmortem coronary angiography has been used in forensic medicine for several decades but its use has never been documented in neonatal hearts. The objective of this case is to report the use of postmortem coronary angiography as a diagnostic modality for neonates suspected to have complex congenital heart anomalies. Case presentation: A 36-week-old female infant required extracorporeal membranous oxygenation for persistent hypotension on day 1 of life. A congenital echocardiogram (ECHO) on day 3 of life revealed multiple anomalous vascular structures within the interventricular septum. The infant passed away on day 4 of life after the parents elected to withdraw support. A consent for autopsy was taken from the parents and a postmortem coronary angiography was performed. The coronary vessels were injected with Iodixanol contrast via a 24 G angiocath under fluoroscopy. The anomalous septal vessels were identified as dilated coronary artery and vein. No other anomalies were identified. Conclusion: Postmortem coronary angiography complements other imaging procedures in understanding the nature of some complex congenital heart defects and in determining the cause of death in such neonates.

Surgical ligation of a patent ductus arteriosus (PDA) is a commonly performed procedure. Complications are infrequent and most commonly include recurrent laryngeal nerve injury and rarely ligation of left pulmonary artery. We report a case of accidental ligation of the descending thoracic aorta leading to a clinically significant coarctation.

Right ventricular (RV) hypoplasia may develop secondary to pulmonary or tricuspid valve atresia. These patients are usually symptomatic early in life and need prompt intervention. Isolated RV hypoplasia is a rare congenital heart disease. We report a case of 23-year-old twins who have been monitored for the last 14 years for isolated right ventricular hypoplasia. ECHO and MRI studies showed a small, heavily trabeculated, nonapex-forming RV and mild tricuspid valve insufficiency. The girl has a patent foramen ovale (PFO). Otherwise, the cardiac anatomy and function was normal. They have both been completely asymptomatic from the cardiac standpoint. The family history is remarkable for death of father at the age of 30 years with autopsy suggestive of a hypoplastic RV. The paternal uncle also died at the age of 46 years, and his son has an unidentified congenital heart disease. The family history appears to suggest an autosomal dominant pattern of inheritance with variable expressivity. However, the chromosome microarray analysis of the twins did not identify any variations of clinical significance.