Aorto-Left Ventricular Tunnel-An Uncommon Entity.

The aorto-left ventricular tunnel is an extracardiac communication that has a specific morphological feature. It is important to differentiate this entity from other diagnoses because the treatment options differ significantly and better outcomes are obtained with this entity.

Left cardiac sympathetic denervation in children with Jervell Lange-Nielsen syndrome and drug refractory torsades - A case series.

INTRODUCTION: Long QT syndrome is an inherited malignant channelopathy which leads to life-threatening arrhythmia, with multiple genotypes. Jervell and Lange-Nielsen syndrome (JLNS) is an autosomal recessive subtype of this disease, characterized by congenital sensorineural deafness and a high incidence of sudden cardiac death (SCD). METHODOLOGY: We prospectively followed up six children who underwent left cardiac sympathetic denervation (LCSD) for JLNS in view of high-risk features despite being on maximally tolerated doses of oral propranolol. RESULTS: Mean age at diagnosis was 2.75 ± 0.39 years, with a significant delay between onset of symptoms and diagnosis (mean 7.2 ± 3.5 months). All had sensorineural hearing loss, conforming to the JLNS phenotype. Mean QTc interval was 603 ± 93 ms, with T wave alternans (TWA) seen in all cases. All were started on propranolol and subsequently subjected to LCSD, and 3 underwent AAI permanent pacemaker implantation. Over a mean follow-up of 20 months, there was a significant reduction in QTc (603 ± 93 ms to 501 ± 33 ms, p = .04), which was persistent on follow-up (525 ± 41 ms) and only two out of six had persistent T wave alternans on ECG (p < .01). None of these children had presyncope, syncope, seizures, torsades de pointes, cardiac arrest or death on follow up following LCSD. CONCLUSION: Jervell Lange-Nielsen syndrome is a subtype of LQTS with high-risk features. LCSD, an effective therapeutic option for those having symptoms despite being on propranolol, results in significant reduction of QTc interval and amelioration of symptoms.

Glass softening in the limit of high heating rates: Heterogeneous devitrification kinetics on nano, meso, and micrometer scale.

When heated rapidly, glasses often devitrify heterogeneously, i.e., by a softening front that originates at the surface of an amorphous film. Yet the fundamentals of this devitrification regime are not completely understood; depending on experimental conditions, the reported front propagation distances differ by an order of magnitude. Using a high-resolution fast scanning calorimetry technique, we have investigated the softening of glassy methylbenzene films with thicknesses between 30 and 1400 nm. We confirm first that, in all films, the devitrification process begins with the formation of a softening front that propagates through the films over distances of ∼50 nm and that the front propagation kinetics at this stage follow an Arrhenius law. However, we also show that, in films with thicknesses above 165 nm, the front propagation does not terminate with the onset of bulk softening. Specifically, increasing the films' thicknesses above 165 nm yields sharp, clearly discernible endotherms that precede the bulk softening endotherms and that are consistent with a two-fold increase in the enthalpic barrier to front propagation at a well-defined critical temperature. We term this phenomenon "Arrhenius discontinuity" and use reaction rate and continuum front dynamics theories to explain its origins and the physical nature of the resulting distinct heterogeneous devitrification processes. Finally, we discuss the findings in the context of recent theoretical, computational, and experimental studies of heterogeneous devitrification by other research groups.

Sudden-Onset Blindness in a Child With Hypertensive Emergency: Unmasking of Chronic Kidney Disease.

A 10-year-old male presented with sudden-onset diminution of vision in both eyes. On systemic examination, he had severe hypertension, no pulse deficit, short stature, and no other focal neurological deficit. Dilated fundoscopy showed bilateral grade 4 hypertensive retinopathy with macular star formation. Detailed laboratory investigations revealed a stage 5 chronic kidney disease (CKD). We present this case to highlight this rare ocular manifestation of CKD in pediatric age group.

Pulmonary artery aneurysm: Harbinger of an ominous disease.

An 11-year-old girl presented with chronic cough and recurrent hemoptysis. On examination, she had features of right heart failure and cyanosis, with severe pulmonary hypertension on echocardiogram. Computed tomography pulmonary angiography showed aneurysmal dilatations of the pulmonary artery with elevated erythrocyte sedimentation rate and C-reactive protein and positive human leukocyte antigen B51. A diagnosis of Hughes-Stovin syndrome (vascular variant of Behcet's syndrome) was confirmed, and she was started on immunosuppression, on which there was improvement.

Thrombosis of aneurysmal pulmonary arteries in patent ductus arteriosus with Eisenmenger syndrome.

A 45-year-old lady with patent ductus arteriosus with Eisenmenger's syndrome had presented with hemoptysis. Computed tomography revealed aneurysmally dilated pulmonary arteries with a large calcified organized thrombus.

Recurrent pericardial effusion in a boy: A clue to underlying pericardial and pulmonary lymphangiectasia.

Pulmonary lymphangiectasia is a rare congenital malformation of lymphatic vessels. We report the case of a 5-year-old boy with recurrent pericardial effusion which was diagnosed to be due to pericardial and pulmonary lymphangiectasia.

Cardiac magnetic resonance imaging in right ventricular outflow tract arrhythmia: a retrospective analysis from a tertiary care centre in South India.

INTRODUCTION: Arrhythmia arising from right ventricular outflow tract (RVOT) is the most common cause of idiopathic ventricular arrhythmia. Previous studies involving cardiac magnetic resonance imaging (MRI) in these patients are known to show abnormalities in up to 60% of patients. METHODOLOGY: This was a retrospective descriptive analysis of patients having idiopathic RVOT arrhythmia who underwent cardiac MRI between January 2010 and December 2020. Clinical and demographic details were recorded from the electronic medical records and cardiac MRI of all patients were reviewed. RESULTS: Among 214 patients with RVOT arrhythmia, 64 underwent cardiac MRI. A total of 41 patients, who did not have any abnormality on baseline echocardiogram were included in the study. There was male preponderance (56.1%), with median age of 43 years. About 43.9% had syncope. Twenty-four-hour Holter study revealed a premature ventricular complex (PVC) burden of 26.3 ± 11.7%. MRI showed structural abnormalities in 51.2% of patients, which commonly included RV sacculations and aneurysms. Late gadolinium enhancement (LGE) was seen in six patients, which was mostly seen in RV free wall. Right ventricular (RV) systolic dysfunction was evident in 29.3%. About 9.8% fulfilled the criteria for arrhythmogenic RV cardiomyopathy (ARVC). Out of 23 patients with baseline abnormalities on ECHO, 87% had structural abnormalities on MRI, and 43.5% fulfilled the criteria for ARVC. CONCLUSIONS: Additional imaging by cardiac MRI helps to identify structural abnormalities in 51.2% of patients with RVOT arrhythmias, even with normal baseline echocardiogram and electrocardiogram (ECG). It is useful tool to rule out ARVC in this subset of patients, and can help in increasing the diagnostic yield in the early stages.

Cerebral Sinovenous Thrombosis due to Hypernatremic Dehydration in a Neonate.

Cerebral sinus venous thrombosis is an uncommon complication of hypernatremic dehydration in neonates. Non-improvement in neurological status even after correction of hypernatremia should lead to suspicion of intracranial complications due to hypernatremia or its overtreatment. Slow correction of hypernatremia, calculated fluid administration, and anticoagulation improve outcome in neonates with CSVT.

Left ventricular noncompaction in primary systemic carnitine deficiency: A rare association.

Left ventricular noncompaction (LVNC) is a rare phenotype of dilated cardiomyopathy. We report a child with primary systemic carnitine deficiency having associated LVNC.

Ventricular septal defect with pulmonary arterial hypertension in an infant: Is there something more than what meets the eye?

An 11-month-old girl was detected to have muscular ventricular septal defect with hyperkinetic pulmonary hypertension (PH) was urgently operated upon. On follow-up, her PH worsened, resulting in right ventricular dysfunction and was later detected to have absent portal vein.

'Misplaced' central venous catheter in a newborn: Reminder of a common congenital anomaly.

Left superior vena cava is the commonest congenital anomaly of systemic venous return. We present the case of a newborn where left jugular venous placement of central venous catheter resulted in cannulation of the left superior vena cava.

Kawasaki disease in siblings in close temporal proximity to each other-what are the implications?

Kawasaki disease (KD) is the commonest medium vessel vasculitis in children. The etiology of KD remains an enigma despite extensive research. Infections are considered to be one of the triggers for KD, especially in genetically susceptible hosts. KD occurring within a short time interval among siblings is an important clinical observation supporting this hypothesis. In addition, siblings of children with KD are at a higher risk of developing the disease as compared with other children. Screening for KD in febrile siblings, therefore, seems prudent. This would help initiate timely therapy and prevent complications. We briefly review 16 English language reports of KD in siblings diagnosed within 1 month of each other to highlight its etiological and therapeutic implications. Key Points • KD should be suspected in febrile children who have a sibling recently diagnosed with KD. • Etiological studies should also focus on siblings who develop KD in close temporal proximity.

Extensive spontaneous cerebral haemorrhage after Russell's viper bite.

A 30-year-old man presented with altered sensorium following Russell's viper bite, which was found to be secondary to intracranial hemorrhage secondary to venom-induced consumptive coagulopathy. He was managed conservatively with blood component transfusion and antivenom injection, and successfully discharged.

Clinical profile of symptomatic congenital cytomegalovirus infection: cases from a tertiary hospital in north India.

We retrospectively analysed the records of nine infants with polymerase chain reaction proven congenital cytomegalovirus (CMV) infection, of which 66% were born preterm. Microcephaly was a universal finding, followed by hepatosplenomegaly in 89%, while chorioretinitis was seen in only 44% cases. The mean age at diagnosis was 3.5 months. Neuroimaging was abnormal in 78%, with ventriculomegaly being the most common finding followed by T2/FLAIR white matter abnormalities, periventricular cysts and intracranial haemorrhage.

Overdosage of Calcarea phosphorica causes severe hypocalcaemia in a toddler.

A four-year-old girl presented with accidental ingestion of 200 tablets of Calcarea phosphorica. Although she was asymptomatic, she was found to have marked hypocalcaemia with a prolonged QTc interval on electrocardiogram. She was successfully treated with intravenous calcium, followed by oral maintenance.

Pituitary macroadenoma secondary to Hashimoto's thyroiditis: inadvertent diagnosis in a pre-pubertal girl.

Pituitary hyperplasia as a result of autoimmune thyroiditis has been rarely reported in children. We report a prepubertal girl with Hashimoto's thyroiditis who was inadvertently diagnosed to have a pituitary macroadenoma based on neuroimaging.

Chylothorax in a Child with Nephrotic Syndrome.

Chylothorax is an uncommon presentation of venous thrombosis in nephrotic syndrome. We present a case of an 8-year-old boy with nephrotic syndrome who presented with prolonged respiratory difficulty and dry cough. A detailed evaluation revealed left chylothorax secondary to thrombosis of the left brachiocephalic vein. He improved with conservative management including anticoagulation therapy, intercostal chest tube drainage, and dietary modification. This case highlights the need to consider venous thrombosis as a cause of chylothorax in patients with nephrotic syndrome to institute appropriate treatment.