Standardization of the diagnostic routine for children with congenital heart disease associated with pulmonary arterial hypertension (PAH-CHD) is crucial, in particular since inappropriate assignment to repair of the cardiac lesions (e.g., surgical repair in patients with elevated pulmonary vascular resistance) may be detrimental and associated with poor outcomes. Thus, members of the Congenital Heart Disease and Pediatric Task Forces of the Pulmonary Vascular Research Institute decided to conduct a survey aimed at collecting expert opinion from different institutions in several countries, covering many aspects of the management of PAH-CHD, from clinical recognition to noninvasive and invasive diagnostic procedures and immediate postoperative support. In privileged communities, the vast majority of children with congenital cardiac shunts are now treated early in life, on the basis of noninvasive diagnostic evaluation, and have an uneventful postoperative course, with no residual PAH. However, a small percentage of patients (older at presentation, with extracardiac syndromes or absence of clinical features of increased pulmonary blood flow, thus suggesting elevated pulmonary vascular resistance) remain at a higher risk of complications and unfavorable outcomes. These patients need a more sophisticated diagnostic approach, including invasive procedures. The authors emphasize that decision making regarding operability is based not only on cardiac catheterization data but also on the complete diagnostic picture, which includes the clinical history, physical examination, and all aspects of noninvasive evaluation.

The Centralized pan-Middle East Survey on the undertreatment of hypercholesterolemia (CEPHEUS) survey evaluated the attainment of low-density lipoprotein cholesterol (LDL-C) goals among patients on lipid-lowering drugs (LLDs) according to the updated National Cholesterol Education Program (NCEP)-Adult Treatment Panel (ATP-III) guideline. The survey was conducted in 6 Arabian Gulf countries. Patients aged ≥18 years on LLDs for at least ≥3 months (stable medication for ≥6 weeks) were recruited. Fasting blood samples were collected at a single visit. In this survey, 5276 (58.2% male) patients were included in the final analysis. The LDL-C goal was attained in 91.1% of low-risk, 52.7% of high-risk, and 32.0% in very-high-risk categories. Goal attainment was directly related to female gender, age<40 years, history of diabetes, and family history of cardiovascular disease. The results of this survey highlight the suboptimal management of hypercholesterolemia across Arabian Gulf countries.

Cardiac catheterization is important in the diagnosis and risk stratification of pulmonary hypertensive vascular disease (PHVD) in children. Acute vasoreactivity testing provides key information about management, prognosis, therapeutic strategies, and efficacy. Data obtained at cardiac catheterization continue to play an important role in determining the surgical options for children with congenital heart disease and clinical evidence of increased pulmonary vascular resistance. The Pediatric and Congenital Heart Disease Task Forces of the Pulmonary Vascular Research Institute met to develop a consensus statement regarding indications for, conduct of, acute vasoreactivity testing with, and pitfalls and risks of cardiac catheterization in children with PHVD. This document contains the essentials of those discussions to provide a rationale for the hemodynamic assessment by cardiac catheterization of children with PHVD.

The association between congenital major urinary tract anomalies (CMUTA) and spontaneous pneumothorax in term newborn infants (SPTNI) is controversial. We conducted a case-control study to test the hypothesis that SPTNI is associated with CMUTA. We compared 80 term infants with spontaneous pneumothorax to 80 healthy control infants. We recorded risk factors, clinical course, therapy, and outcome. Only 1 infant of 60 infants (1.7%) had CMUTA with SPTNI, as revealed by renal ultrasound studies. This is comparable to the 1.4% rate reported for CMUTA in healthy newborn infants by Steinhart et al. ([1988] Pediatrics 82:609-614). SPTNI were significantly more likely in males with higher birth weights and with vacuum delivery. Sixty-seven (84%) infants with SPTNI had follow-up for a mean and median of 46.4 and 39 months, respectively (range, 1-126 months), without manifesting any renal or pulmonary complications.

Aim:Infantile scimitar syndrome is a rare condition, with most of the literature reports being limited to case reports and a few case series. The aim of this study was to review patients with infantile scimitar syndrome who presented to our hospital from July 2000 to January 2011.Materials and Methods:In this retrospective study, we evaluated the medical records of patients aged 0–14 years who were symptomatic before the age of 1 year and were subsequently diagnosed with the syndrome. A total of 16 patients with the infantile form of scimitar syndrome were identified from the database.Results:The median age at presentation was 14 days, with a median age at diagnosis of 55 days. Fifty-six percent of the patients were females. Tachypnea was the major presenting symptom and 13 out of 16 patients had pulmonary hypertension. Of the 13 patients with pulmonary hypertension, 7 had systemic collaterals, which were treated by coil occlusion together with medications, and 3 had corrective surgery. The mortality rate was 3/16 (18.8%) over the 10.5 years study period.Conclusions:Infantile scimitar syndrome is a rare congenital anomaly that needs a high degree of suspicion for early referral and management. The association of the syndrome with pulmonary hypertension leads to recurrent and prolonged hospitalization.