Comparison of intravenous sildenafil with inhaled nitric oxide for acute vasodilator testing in pulmonary arterial hypertension.

Acute vasodilator testing (AVT) identifies acute responders for initiation of calcium channel blockers in pulmonary arterial hypertension (PAH) and operability in congenital heart disease (CHD). We sought to determine the feasibility of intravenous sildenafil (ivS) as an alternative to inhaled nitric oxide (iNO) in AVT. All patients with PAH undergoing cardiac catheterization for AVT (November 2015 to December 2020) were prospectively enrolled. Hemodynamic data were obtained at baseline, with iNO 20 ppm and ivS (0.25 mg/kg for children and 10 mg for adults). We studied 44 patients with a mean age of 20.5 ± 14.4 years (27 [61%] females and 20 [45%] children). There were 17 (38.6%) patients in the CHD group for operability assessment and 27 patients in non-CHD group (idiopathic pulmonary arterial hypertension-16 [36.3%], residual PAH after shunt closure-7 [15.9%], and 2 cases [4.5%] each of familial PAH and portopulmonary hypertension). There was an excellent intraclass correlation for mean pulmonary artery pressures (0.903, 95% confidence interval, CI: 0.809-0.949, p < 0.001), mean aortic pressures (0.745, 95% CI: 0.552-0.858, p < 0.001), pulmonary vascular resistance index (0.920, 95% CI: 0.858-0.956, p < 0.001), systemic vascular resistance (SVR) index (0.828, 95% CI: 0.706-0.902, p < 0.001), and the ratio of pulmonary and SVR indices (0.857, 95% CI: 0.752-0.919, p < 0.001) between the two agents. There were two responders, both in non-CHD group, and were identified by iNO and ivS. The hemodynamic effects of ivS show excellent correlation with iNO and could be a potential alternative agent for identifying acute responders during AVT.

Percutaneous ultrasound guided thrombin injection for axillary artery pseudoaneurysm following stenting of the arterial duct in two infants: Case report with review of literature.

We report pseudoaneurysm of the axillary artery following stenting of the arterial duct in two infants. They were both successfully managed by ultrasound guided thrombin injection with preservation of native arterial flow. We also review the relevant literature.

Modified technique for uncommon coarctation of the aorta with arch hypoplasia.

A true presubclavian coarctation of the aorta with arch hypoplasia without major intracardiac anomaly can result in a surgical dilemma. Theoretically, one can avoid repair through median sternotomy using selective cerebral perfusion and its sequelae since no major intracardiac repair is needed. Repair through thoracotomy is technically challenging if arch reconstruction is required and precise surgical planning is required to avoid spinal hypoperfusion and to maintain cerebral perfusion. Moreover, inadequate repair often has resulted in hypoplastic aortic arch or residual coarctation on follow-up requiring future intervention. We have employed a modified surgical repair through thoracotomy avoiding cardiopulmonary bypass to address presubclavian coarctation of the aorta with diffuse arch hypoplasia with theoretically less chance for future stenosis.

Transcatheter closure of a membranous ventricular septal defect in a 1.8-kg infant using Amplatzer Duct Occluder II additional size device.

Catheter closure of membranous ventricular septal defects is generally not considered feasible in small infants. We report the successful closure of a membranous ventricular septal defect in a 1.8-kg infant with bilateral femoral artery occlusion using Amplatzer Duct Occluder II additional size device. The ventricular septal defect was crossed from the right ventricle, and the device was deployed using transthoracic echocardiographic guidance.

Echocardiographic screening for rheumatic heart disease in high and low risk Australian children.

BACKGROUND: Echocardiographic screening for rheumatic heart disease (RHD) is becoming more widespread, but screening studies to date have used different echocardiographic definitions. The World Heart Federation has recently published new criteria for the echocardiographic diagnosis of RHD. We aimed to establish the prevalence of RHD in high-risk Indigenous Australian children using these criteria and to compare the findings with a group of Australian children at low risk for RHD. METHODS AND RESULTS: Portable echocardiography was performed on high-risk Indigenous children aged 5 to 15 years living in remote communities of northern Australia. A comparison group of low-risk, non-Indigenous children living in urban centers was also screened. Echocardiograms were reported in a standardized, blinded fashion. Of 3946 high-risk children, 34 met World Heart Federation criteria for definite RHD (prevalence, 8.6 per 1000 [95% confidence interval, 6.0-12.0]) and 66 for borderline RHD (prevalence, 16.7 per 1000 [95% confidence interval, 13.0-21.2]). Of 1053 low-risk children, none met the criteria for definite RHD, and 5 met the criteria for borderline RHD. High-risk children were more likely to have definite or borderline RHD than low-risk children (adjusted odds ratio, 5.7 [95% confidence interval, 2.3-14.1]; P<0.001). CONCLUSIONS: The prevalence of definite RHD in high-risk Indigenous Australian children approximates what we expected in our population, and no definite RHD was identified in the low-risk group. This study suggests that definite RHD, as defined by the World Heart Federation criteria, is likely to represent true disease. Borderline RHD was identified in children at both low and high risk, highlighting the need for longitudinal studies to evaluate the clinical significance of this finding.

Decline in arterial partial pressure of oxygen after exercise: a surrogate marker of pulmonary vascular obstructive disease in patients with atrial septal defect and severe pulmonary hypertension.

OBJECTIVES: To examine the utility of decline in arterial partial pressure of oxygen after exercise as a marker of pulmonary vascular obstructive disease in patients with atrial septal defect and pulmonary hypertension. METHODS: Treadmill exercise was performed in 18 patients with atrial septal defect and pulmonary hypertension. Arterial blood gas samples were obtained before and after peak exercise. A decline in the arterial pressure of oxygen of more than 10 millimetres of mercury after exercise was considered significant based on preliminary tests conducted on the controls. Cardiac catheterisation was performed in all patients and haemodynamic data sets were obtained on room air, oxygen, and a mixture of oxygen and nitric oxide (30-40 parts per million). RESULTS: There were 10 patients who had more than a 10 millimetres of mercury drop in arterial partial pressure of oxygen after exercise and who had a basal pulmonary vascular resistance index of more than 7 Wood units per square metre. Out of eight patients who had less than a 10 millimetres of mercury drop in arterial partial pressure of oxygen after exercise, seven had a basal pulmonary vascular resistance index of less than 7 Wood units per square metre, p equals 0.0001. A decline in arterial partial pressure of oxygen of more than 10 millimetres of mercury predicted a basal pulmonary vascular resistance index of more than 7 Wood units per square metre with a specificity of 100% and a sensitivity of 90%. CONCLUSIONS: A decline in arterial partial pressure of oxygen following exercise appears to predict a high pulmonary vascular resistance index in patients with atrial septal defect and pulmonary hypertension. This test is a useful non-invasive marker of pulmonary vascular obstructive disease in this subset.

Dynamics of growth and weight transitions in a pediatric cohort from India.

BACKGROUND: There is paucity of information regarding time trends of weight status in children from rapidly developing economies like India. The aim of the study was to analyse the dynamics of growth and weight transitions in a cohort of school children from India. METHODS: A population of 25 228 children was selected using stratified random sampling method from schools in a contiguous area in Ernakulam District, Kerala, India. Weight and height were measured at two time points, one in 2003-04 and another in 2005-06. The paired data of 12 129 children aged 5-16 years were analysed for the study. RESULTS: The mean interval between the two surveys was 2.02 +/- 0.32 years. The percentage of underweight, normal weight, overweight and obese children in the year 2003-04 were 38.4%, 56.6%, 3.7%, and 1.3% respectively. The corresponding figures in year 2005-06 were 29.9%, 63.6%, 4.8% and 1.7% respectively. Among the underweight children, 34.8% migrated to normal weight status and 0.1% migrated to overweight status. Conversion of underweight to normal weight predominated in urban area and girls. Among the normal weight children, 8.6% migrated to underweight, 4.1% migrated to overweight and 0.4% migrated to obesity. Conversion of normal weight to overweight status predominated in urban area, private schools and boys. Conversion of normal weight to underweight predominated in rural area, government schools and boys. Among the overweight children, 26.7% migrated to normal weight status, 16.4% became obese and 56.9% retained their overweight status. Of the obese children, 6.2% improved to normal weight status, 25.3% improved to overweight status and 68.5% remained as obese in 2005-06. There was significant difference in trends between socio demographic subgroups regarding conversion of underweight status to normal weight as well as normal weight status to overweight. CONCLUSION: The study population is experiencing rapid growth and nutritional transitions characterised by a decline in the underweight population coupled with an escalation of the overweight population. The heterogeneous nature of this transition appears to be due to differences in socio demographic factors.

Duplication of the tension apparatus of the tricuspid valve.

We report an infant with a large perimembranous ventricular septal defect, who had two separate orifices in the tricuspid valve, each supported by separate cordal apparatus, detected incidentally during surgery.