Dobutamine infusion for complex congenital heart disease with pulmonary hypertension in an infant posted for open pyloromyotomy

Ravindra, Madhavi; Bhagya, D

doi:10.4103/2394-6954.163092

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“…However, to the best of our knowledge, there are only five previously reported cases in the literature on the occurrence of both conditions in a patient [10–12]. The characteristics of those cases of HPS and PPHN or PH are summarized in Table 1 [10–13]. Brouwers et al considered that if there was no common pathophysiologic pathway between HPS and PPHN, the combination of both conditions would occur in approximately 1 per 100,000 live births, and they proposed a deficiency in NO as a common pathway [10].…”

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confidence: 99%

Hypertrophic pyloric stenosis following persistent pulmonary hypertension of the newborn: a case report and literature review

et al. 2018

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BackgroundAlthough persistent pulmonary hypertension of the newborn (PPHN) and infantile hypertrophic pyloric stenosis (HPS) are both well-known diseases that occur in early infancy, PPHN complicated by HPS is rare. As nitric oxide (NO) is an important mediator of biological functions, on both the vascular endothelium and smooth muscle cells, the decreased production of NO might play a role in the pathogenesis of both PPHN and HPS. We present the case of a neonate who developed HPS following PPHN, including a detailed review on research published to date, and we discuss the pathogenesis of PPHN and HPS.Case presentationA female neonate born at 38 weeks of gestation, weighing 3140 g, developed PPHN due to meconium aspiration syndrome. Intensive treatment with high frequency oscillations and inhaled NO were initiated, and sildenafil and bosentan were added. She gradually recovered. At 15 days of age, the patient developed recurrent vomiting after feeding and the diagnosis of HPS was made. Intravenous atropine therapy was started at 20 days of age, but the efficacy was clinically unsatisfactory. The coadministration with transdermal nitroglycerin improved the symptoms, and oral feeding was successfully re-introduced.ConclusionsOur patient recovered from both PPHN and HPS using NO-related medications. A decrease in NO synthesis is likely to be a common pathway for PPHN and HPS.

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