Right ventricular-vascular coupling ratio in pediatric pulmonary arterial hypertension: A comparison between cardiac magnetic resonance and right heart catheterization measurements.

BACKGROUND: In pulmonary arterial hypertension (PAH), right ventricular (RV) failure is the main cause of mortality. Non-invasive estimation of ventricular-vascular coupling ratio (VVCR), describing contractile response to afterload, could be a valuable tool for monitoring clinical course in children with PAH. This study aimed to test two hypotheses: VVCR by cardiac magnetic resonance (VVCRCMR) correlates with conventional VVCR by right heart catheterization (VVCRRHC) and both correlate with disease severity. METHODS AND RESULTS: Twenty-seven patients diagnosed with idiopathic and associated PAH without post-tricuspid shunt, who underwent RHC and CMR within 17 days at two specialized centers for pediatric PAH were retrospectively studied. Clinical functional status and hemodynamic data were collected. Median age at time of MRI was 14.3 years (IQR: 11.1-16.8), median PVRi 7.6 WU × m2 (IQR: 4.1-12.2), median mPAP 40 mm Hg (IQR: 28-55) and median WHO-FC 2 (IQR: 2-3). VVCRCMR, defined as stroke volume/end-systolic volume ratio was compared to VVCRRHC by single-beat pressure method using correlation and Bland-Altman plots. VVCRCMR and VVCRRHC showed a strong correlation (r = 0.83, p < 0.001). VVCRCMR and VVCRRHC both correlated with clinical measures of disease severity (pulmonary vascular resistance index [PVRi], mean pulmonary artery pressure [mPAP], mean right atrial pressure [mRAP], and World Health Organization functional class [WHO-FC]; all p ≤ 0.02). CONCLUSIONS: Non-invasively measured VVCRCMR is feasible in pediatric PAH and comparable to invasively assessed VVCRRHC. Both correlate with functional and hemodynamic measures of disease severity. The role of VVCR assessed by CMR and RHC in clinical decision-making and follow-up in pediatric PAH warrants further clinical investigation.

Invisible friends across four countries: Kenya, Malawi, Nepal and the Dominican Republic.

Cross-cultural comparisons of the prevalence of invisible/imaginary companions are difficult due to the use of various methods of data gathering and the lack of sampling in developing countries. The present study took place among 443 children (3-8-year-olds) in four different countries (Kenya, Malawi, Nepal and the Dominican Republic) employing the same interview method. Among all the children 21% affirmed that they had invisible/imaginary companions at the time of the interview. But the rates between countries varied significantly from a low of 5% in Nepal to a high of 34% in the Dominican Republic. The results suggest that the potential for the phenomenon transcends cultural particularity even as culture plays an important role for supporting or discouraging invisible/imaginary companions.

Chemotherapy and its effect on testicular morphology in children.

Bilateral testicular biopsies were performed on 25 boys between 4 and 16 years old with the diagnosis of acute lymphocytic leukemia after the induction of regression with vincristine and prednisone, followed by cyclic maintenance therapy with a combination of 6-mercaptopurine, methotrexate, L-asparaginase, cyclophosphamide and doxorubicin for 2 years. These drugs were given in accordance with children's cancer study group protocols. Only 3 boys had positive biopsies (12 per cent), although post-treatment testicular morphology was abnormal in the overwhelming majority. Interstitial fibrosis was present in 16 children (70 per cent), basement membrane thickening in 12 (50 per cent) and, most importantly, a reduction in the tubular fertility index of greater than 50 per cent in 20 (80 per cent). There was no relationship between the abnormal morphology encountered and the age of the patient when treated. Most of these changes may be reversible with time but only a biopsy at a later date can establish the long-term effects of treatment on testicular morphology and, perhaps, help to predict future fertility potential.

Absence of the aortic valve: antenatal and postnatal two-dimensional and Doppler echocardiographic features.

Antenatal and postnatal two-dimensional and gated pulsed Doppler echocardiography, beginning at 32 weeks' gestation, were used to evaluate the cardiovascular contribution to nonimmunologic hydrops in a gravida 1, para 0 nineteen year old woman. Antenatal two-dimensional imaging demonstrated a hypoplastic left heart variant of complete common atrioventricular (AV) canal. Antenatal gated pulsed Doppler examination of the fetal thoracoabdominal aorta revealed atypical pandiastolic retrograde flow. Postnatal noninvasive examination at 36 weeks' gestational age was unchanged from the prenatal study. Postmortem examination confirmed the noninvasive anatomic findings and revealed polysplenia. The mechanism of pandiastolic retrograde flow was attributable to "absence of the aortic valve," a previously unreported congenital cardiac anomaly, which resulted in severe antenatal and postnatal aortic regurgitation.

The significance of cystic hygroma in fetuses.

Cystic hygroma of the neck was observed in seven spontaneously aborted, severely autolyzed female fetuses. Four of five karyotyped cases had the 45,XO karyotype; one was a normal female, 46,XX. Diagnostic features associated with monosomy X in fetuses, which were observed in six of these cases, include large cystic hygromas, generalized edema, edematous chorionic villi with scalloped borders and trophoblastic inclusions, and, possibly, a small heart. In contrast, the single euploid fetus in this series had a small cystic hygroma, no hydrops, and chorionic villi of normal size. These features may differentiate cystic hygromas associated with the XO karyotype, which are not associated with recurrence risk, from those associated with a familial syndrome.

Acute necrotizing enterocolitis in infancy: a review of 64 cases.

Sixty-four cases of necrotizing enterocolitis are reviewed. The diagnosis was based on tissue examination in 57 and on the clinical syndrome, including pneumatosis, in 7. Three factors are important in the development of the disease: injury to the intestinal mucosa, bacteria, and feedings. The indications for surgical intervention are pneumoperitoneum, signs of peritonitis, and intestinal obstruction. The importance of stress in the etiology of the disease is confirmed by the high incidence of perinatal complications, particularly hypoxia. The mortality was high, but results are improving with the institution of early aggressive treatment.