RESUMO
The optimal approach for supravalvar right ventricular outflow tract obstruction(RVOTO) after arterial switch operation(ASO) is unclear. The results of percutaneous balloon dilatation have been variable. We report the results of simultaneous double balloon dilation for RVOTO after ASO. Sixteen patients (1.3(0.7-3.8) years; 9.8(8.1-15.1) kgs underwent the procedure at 14(8-44.5) months after ASO. Salient technical features included placement of balloons over stiff guide-wires positioned in both branch pulmonary arteries to enable dilation of the distal-most main pulmonary artery (MPA) with high inflation pressures (~ 12-14 atmospheres) and short inflation-deflation cycles. Effective balloon size was based on the PA annulus or MPA distal to the narrowing. The final balloon: narrowest segment diameter ratio was 2.7. Following dilation, the right ventricle to systemic systolic pressure ratio decreased from 0.9 ± 0.18 to 0.52 ± 0.16 (p < 0.001) and mean RVOT gradient from 78 ± 18 to 34 ± 13.9 mmHg (p < 0.001). Narrowest diameter improved from 5.4 ± 2.2 to 9.2 ± 2.2 mm. There were no major complications. Two patients with inadequate relief (final RV-systemic ratios: 1.03 and 0.7) were referred for surgery. At median follow up of 9 months, IQR 7-22, range 5-73, others are free of re interventions with median RVOT gradient of 42, IQR 27-49, range 21-55 mmHg. The immediate and short-term follow up results of double balloon dilatation for supravalvar RVOTO is encouraging and may avoid the need for repeat surgery in the majority of patients. Further follow up is needed to determine the long-term durability of the results.
RESUMO
We report a case with prenatal diagnosis of transposition of great arteries (TGA) with L-posed aorta (SDL-TGA) which was confirmed by postnatal echocardiography. The anatomic findings were confirmed during the successful arterial switch operation (ASO). The technical challenges of ASO in the L-posed aorta are also described.
RESUMO
BACKGROUND: It is widely perceived that the value of physical examination in paediatric cardiology has diminished with the increasing availability of echocardiography. The accuracy of physical examination of cardiovascular system in children has not been systematically tested. METHODS: This is a cross-sectional, diagnostic accuracy study from the paediatric cardiology clinic of a tertiary referral hospital in South India. A total of 545 children with 5 common cardiac conditions were included-normal heart, atrial septal defect, patent ductus arteriosus, ventricular septal defect (VSD) and VSD with pulmonic stenosis. Physical examination was documented by a paediatric cardiology fellow and a consultant who were blinded to previous investigations and to each other. The accuracy of physical examination of the fellow and the consultant was determined for each patient group by comparing with echocardiography. Interobserver agreement was calculated using kappa statistics. RESULTS: Physical examination differentiated normal hearts from abnormal with an accuracy of 95.0% for fellows and 96.3% for consultants. For all abnormal hearts, the results for fellows and consultants, respectively, were as follows: sensitivity: 94.3%, 94.9%, specificity: 96.2%, 98.6%, accuracy: 95.0%, 96.3%, positive likelihood ratio: 24.8, 66.4 and negative likelihood ratio: 0.06, 0.05. There was good agreement between fellows and consultant for all patient groups (kappa: 0.72-1), except for large VSD (kappa: 0.232). Younger age and haemodynamically insignificant lesions were associated with incorrect diagnosis. CONCLUSION: This study underscores the utility of clinical examination in initial screening for commonly encountered congenital cardiac conditions even in the current era of echocardiography.
Assuntos
Sistema Cardiovascular , Cardiopatias Congênitas , Comunicação Interventricular , Criança , Estudos Transversais , Cardiopatias Congênitas/diagnóstico , Comunicação Interventricular/complicações , Humanos , Exame FísicoRESUMO
Left Ventricular Non Compaction (LVNC) is considered a unique cardiomyopathy according to the American Heart Association guidelines. The genetic ethology of LVNC in children is not completely understood although upto 41% of LVNC are thought to be genetic. We report a family with LVNC due to a novel mutation in the MYH 7 gene.