Persistent Pulmonary Hypertension in Newborns: A Rare and Perplexing Diagnosis - Abstract
Backgrounds: Alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/ MPV) is a rare fatal disease. There are around 200 cases reported in the literature. It is a disorder of immature lobular development and reduced capillary density in the lungs. The pulmonary veins run alongside pulmonary arteries, instead of in the interlobular septa. Mortality to date is nearly 100% Case Report: We performed a retrospective chart review of five cases of ACD/MPV at our center from 2005-2019. We reviewed clinical history, course of hospitalization, echocardiograms, chest imaging including chest CT, histology including autopsy findings, and the medical management. We present five cases from our center. The diagnosis was confirmed by either autopsy or biopsy. All the newborns had severe PPHN refractory to treatment. Conclusion: ACD/MPV should always be considered as a cause of severe persistent pulmonary hypertension refractory to treatment including inhaled nitric oxide, conventional mechanical ventilation, and medical management. It is imperative to have a high index of suspicion in cases of severe refractory pulmonary hypertension and confirm the diagnosis of ACD/MPV as early as possible. Developing and implementing an institutional algorithm for the management of these cases can help to ensure that everyone involved in care of the patient are considering at the big picture including utilization of resources while combating with some very practical challenges.