Cardiovascular outcomes improve in children with renovascular hypertension following endovascular and surgical interventions.

BACKGROUND: Renovascular hypertension (RenoVH) is a cause of hypertension in children. A common cause of RenoVH is renal artery stenosis which acts by reducing blood supply to renal parenchyma and activating the renin-angiotensin-aldosterone axis, often leading to cardiac remodelling. This longitudinal observational study aims to describe occurrence of cardiovascular changes secondary to RenoVH and also any improvement in cardiac remodelling after successful endovascular and/or surgical intervention. METHODS: All patients with RenoVH referred to our centre, who received ≥ 1 endovascular intervention (some had also undergone surgical interventions) were included. Data were collected by retrospective database review over a 22-year period. We assessed oscillometric blood pressure and eight echocardiographic parameters pre- and post-intervention. RESULTS: One hundred fifty-two patients met inclusion criteria and had on average two endovascular interventions; of these children, six presented in heart failure. Blood pressure (BP) control was achieved by 54.4% of patients post-intervention. Average z-scores improved in interventricular septal thickness in diastole (IVSD), posterior Wall thickness in diastole (PWD) and fractional shortening (FS); left ventricular mass index (LVMI) and relative wall thickness (RWT) also improved. PWD saw the greatest reduction in mean difference in children with abnormal (z-score reduction 0.25, p < 0.001) and severely abnormal (z-score reduction 0.23, p < 0.001) z-scores between pre- and post-intervention echocardiograms. Almost half (45.9%) had reduction in prescribed antihypertensive medications, and 21.3% could discontinue all antihypertensive therapy. CONCLUSIONS: Our study reports improvement in cardiac outcomes after endovascular + / - surgical interventions. This is evidenced by BP control, and echocardiogram changes in which almost half achieved normalisation in systolic BP readings and reduction in the number of children with abnormal echocardiographic parameters. A higher resolution version of the Graphical abstract is available as Supplementary information.

Criss-cross heart: Transthoracic echocardiographic features.

OBJECTIVE: To study the echocardiographic features of criss-cross heart (CCH), a congenital cardiac anomaly characterized by crossed ventricular inflow streams, in Indian patients. METHODS: In this retrospective observational study, all pediatric echocardiograms performed in a single tertiary care institution in South India over a three-year period were scrutinized for a diagnosis of CCH. Demographic, clinical and echocardiographic data were collected from patients' medical records and echocardiographic database. Crossed ventricular inflow streams was identified when there was inability to visualize both atrio-ventricular valves in a single imaging plane in cardiac four chamber view. RESULTS: CCH was diagnosed in five patients from 10,500 pediatric echocardiographic studies. The age at diagnosis ranged from one month to 8 years. Cyanosis was present in all but one of the five cases. Crossed ventricular inflow streams was present by definition in all cases, whereas superior-inferior ventricular relationship was present in only three cases. All cases were associated with ventricular septal defects. Atrio-ventricular discordance was seen in three cases and concordance in two. Ventriculo-arterial discordance was seen in three cases, concordance in one and double outlet right ventricle in one. Three cases had pulmonary stenosis and the other two had pulmonary arterial hypertension. Straddling of AV valve was observed in four cases and hypoplastic aortic arch in one case. CONCLUSION: CCH is an extremely rare congenital cardiac anomaly. Superior-inferior ventricular relationship often co-exists with CCH, but is not necessarily present in all cases. CCH requires early diagnosis because of its common association with diverse cardiac anomalies.

Spectrum of pulmonary valve morphology and its relationship to pulmonary trunk in tetralogy of Fallot.

BACKGROUND: Tetralogy of Fallot (TOF) is a complex congenital heart disease with anatomic variations. Although the pulmonary valve in TOF is abnormal, it has not been studied well, especially on newer imaging modalities such as multidetector computed tomography (CT), which gives excellent anatomic detail. AIMS: The aim of this study was to assess the morphology of pulmonary valve in TOF on CT and evaluate its association with the degree of hypoplasia of infundibulum and pulmonary trunk. MATERIALS AND METHODS: The cardiac CT scans of 30 patients with TOF were reviewed to evaluate the morphology of the pulmonary valve, infundibulum, and pulmonary arteries. Fisher's exact test was performed to examine the association between pulmonary valve morphology and degree of hypoplasia of the infundibulum and pulmonary trunk. RESULTS: 16.7% of patients with TOF had pulmonary atresia. The prevalence of tricuspid, bicuspid, and absent valves were 10%, 53.3% and 6.7%, respectively. In another 13.3% of patients, although valve tissue was present, exact morphology could not be determined on CT. The commissures of 62.5% of the bicuspid valves were at 12 o'clock and 6 o'clock or slightly off the midline. There was statistically significant association between valve morphology and degree of infundibular hypoplasia (P < 0.001) and calibre of pulmonary trunk (P < 0.001). CONCLUSION: Morphological abnormality of the pulmonary valve is common in TOF. The most common type of pulmonary valve in TOF patients is bicuspid valve with commissures at 12 o'clock and 6 o'clock or slightly off the midline. Fewer cusps of the pulmonary valve are associated with a more severe degree of pulmonary artery hypoplasia.

Pseudoaneurysm of the left atrium following infective endocarditis.

Transthoracic echocardiogram of a 3-year-old child showed a hypoechoic cavity in the posterior wall of the left atrium communicating with the left ventricle through an orifice in the mitral annulus, suggestive of pseudoaneurysm (Ps), probably the result of infective endocarditis. Three-dimensional echocardiography was helpful to confirm the diagnosis and assess the anatomical relationship of the Ps.

Atrial septal defect with right-to-left shunt in the absence of pulmonary hypertension.

We describe the case of a 27-year-old gentleman who developed late-onset clubbing and cyanosis. Transoesophageal echocardiography revealed a 27-mm ostium secundum atrial septal defect and a large, floppy Eustachian valve directing right atrial blood to the left side of the heart.

Unraveling the Mystery Behind A Patient with 'Refractory Seizures'.

Neurological manifestations such as seizures although rare are well recognized presentations of cardiac arrhythmias. Almost always, such events are primarily generalized in nature leading on to loss of consciousness. Rarely however, cardiac seizures can manifest with focal neurological events. We report a case of a sexagenarian who presented with recurrent focal seizures with secondary generalization, who was misdiagnosed and treated as seizure disorder; only a careful history and focussed investigations helped in realising a precise diagnosis.

Transcatheter closure of ruptured sinus of valsalva to left ventricle.

We report a rare case of ruptured right sinus of valsalva into the left ventricle (LV). Transthoracic echocardiography showed a marked turbulent flow from the right aortic sinus to the LV. We describe a novel technique of closure of this defect with duct occluder, involving the formation of an arterio-arterial loop, without resorting to the usual arteriovenous loop.