The aim of this study is to evaluate quality of life in four domains (physical, emotional, social, and school) in pediatric patients with pulmonary hypertension (PH) using a validated survey (PedsQL). This is a prospective cohort study of pediatric patients aged 2–18 years with PH. Parents of all children and patients aged 8–18 years with appropriate developmental capacity completed the PedsQL survey in the clinic. Results were compared with published norms for pediatric patients, those with congenital heart disease (CHD) and cancer. Thirty-three children were enrolled yielding 32 parent and 18 patient self-reports: seven patients were aged 2–4 years; three were aged 5–7 years; 11 were aged 8–12 years, and 12 were aged 13–18 years. Twenty-one patients were classified as World Health Organization (WHO) Group I pulmonary arterial hypertension (PAH), 11 WHO Group III PH due to lung disease, and one WHO Group V with segmental PH. Thirteen patients were NYHA functional class (FC) 1, 12 were FC 2, eight were FC 3, and none were FC 4. The PH cohort had significantly lower scores than healthy children in all domains on both parent and self-report. The PH cohort also had significantly lower scores than patients with CHD (parent report: total, physical, social, school; patient self-report: total, physical, school) and cancer (parent report: school; patient self-report: physical, school). Close to 50% of participants reported at risk scores in each domain. The quality of life in pediatric PH patients assessed by PedsQL revealed functional impairment in multiple domains . Administration of the PedsQL during outpatient encounters may provide an easy, reproducible method to assess quality of life and direct referral for interventional services.

Patients with pulmonary hypertension associated with congenital heart disease make up an increasing proportion of the total pulmonary hypertension population who bring with them added complexity because of underlying anatomical and hemodynamic abnormalities. Currently, no consensus recommendations are available on how to best manage this group of patients for either the primary cardiologist or pulmonary hypertension subspecialist, including timing of referral. The purposes of this document are (1) to describe the various pulmonary hypertension groups and subgroups associated with congenital heart disease, (2) to describe imaging modalities used in patient evaluation, (3) to elucidate medical and surgical management considerations, (4) to highlight disparities within this population, and (5) to identify gaps and future research needs of patients with pulmonary hypertension associated with congenital heart disease.

Development of pulmonary hypertension after initiation of diazoxide for treatment of neonatal hyperinsulinemic hypoglycemia is a rare, but previously described association. Risk factors for development of diazoxide associated pulmonary hypertension include lower gestational age and congenital heart disease. This novel case report describes an infant with noncompaction cardiomyopathy who developed pulmonary hypertension shortly after initiation of diazoxide for hyperinsulinemic hypoglycemia which resolved upon cessation of the drug. This case highlights the benefit of having pre-treatment knowledge of underlying cardiac anatomy and makes a case for routine echocardiographic screening for neonates initiating diazoxide treatment.

Annual surveillance coronary angiograpyhy to screen for graft coronary vasculopathy is routine practice after orthotopic heart transplantation. Traditionally, this is performed with direct coronary angiography using static single-plane or biplane angiography. Recently, technological advances have made it possible to perform dual-axis rotational coronary angiography (RA). This technique differs from standard static single-plane or biplane angiography in that a single detector is preprogrammed to swing through a complex 80° arc during a single injection. It has the advantage of providing a perspective of the vessels from a full arc of images rather than from one or two static images per contrast injection. The current study evaluated two coronary angiography techniques used consecutively at a single center to evaluate pediatric heart transplant recipients for graft coronary vasculopathy. A total of 23 patients underwent routine coronary angiography using both biplane static coronary angiography (BiP) and RA techniques at the Children's Hospital of Wisconsin from February 2009 to September 2010. Demographic and procedure data were collected from each procedure and analyzed for significance utilizing a Wilcoxon rank sum test. No significant demographic or procedural differences between the BiP and the RA procedures were noted. Specific measures of radiation dose including fluoroscopy time and dose area product were similar among the imaging techniques. The findings show that RA can be performed safely and reproducibly in pediatric heart transplant recipients. Compared with standard BiP, RA does not increase radiation exposure or contrast use and in our experience has provided superior angiographic imaging for the evaluation of graft coronary vasculopathy.