Temporary pacing through umbilical venous route for neonatal heart failure due to complete atrioventricular block.

We present a case of a neonate who presented with worsening heart failure due to congenital complete atrioventricular (AV) block, secondary to maternal anti Ro/SSA and anti-LA/SSB antibodies. The patient was implanted a temporary pacemaker in view of hemodynamic deterioration and subsequently was weaned off ionotropic support and referred for permanent epicardial pacemaker implantation. We report temporary pacemaker implantation in a neonate with hemodynamic instability as a stabilizing measure and discuss technical challenges for the same.

The 'qRBBB myocardial infarction': Unwrapping an old enigma.

This single-center, prospective, observational study was conducted at a tertiary-care center over a span of two years. Patients presenting with acute Anterior-Wall STEMI were included as a study population. The subgroups included qRBBB pattern on ECG and non-qRBBB group. Among 1128 patients included in the study, 100 (11.28 %) patients presented with qRBBB pattern. Increased risk of cardiogenic shock, increased hospital-stay, a higher Killip class on presentation, high incidence of recanalized IRA, remarkably depressed LVEF were significantly associated with qRBBB-MI, which is a menacing form of ACS that leads not only to a high mortality but also to a long-term morbidity.

Anomalous origin of coronary artery from the opposite aortic sinus of Valsalva-a single center experience with a therapeutic conundrum.

BACKGROUND: Coronary artery anomalies are rare congenital abnormalities, most often found incidentally on conventional coronary angiography and CT angiography (CTA). CTA better delineates the origin and course of anomalous coronaries. Anomalous origin of coronary artery from the opposite aortic sinus of Valsalva (ACAOS) has a prevalence of 1% with a very few having an interarterial (malignant) course. There is limited literature, especially in the Indian population, dealing with this topic. METHODS: In this retrospective observational study, angiographic data of 8500 consecutive patients from June 2011 to December 2019 at a large tertiary care hospital in western India was analyzed. Patients diagnosed with ACAOS underwent CTA for delineation of the exact anatomy. Those with a non-malignant course with evidence of ischemia clinically or on stress myocardial perfusion imaging (MPI), underwent PCI. Others with a non-malignant course were medically managed. Patients with malignant (interarterial) course were revascularized by coronary artery bypass graft (CABG) surgery in case of LCA involvement or positive MPI test. Asymptomatic patients with negative MPI were managed medically. Clinical follow-up over 12 months of patients undergoing PCI and those with a malignant course showed no major adverse cardiovascular events (MACE). RESULTS: Of the 8500 patients studied, 74 (0.87%) had ACAOS. Of these, 51 (68.9%) patients had anomalous origin of right coronary artery (RCA) from the left aortic sinus, 21 (28.4%) had anomalous origin of the circumflex artery (Cx) from the right aortic sinus and two patients (2.7%) had an anomalous origin of the left main coronary artery (LCA) from the right aortic sinus. Interarterial course was found in five (6.7%) patients. Of these, four patients underwent CABG-one asymptomatic patient with LCA from right aortic sinus and three with positive MPI in anomalous RCA. All five patients with malignant course and ten patients who underwent PCI were free of MACE over 12 months' clinical follow up. CONCLUSIONS: ACAOS is a rare anomaly and if interarterial course is excluded, then PCI is feasible in selected cases with significant stenosis. Patients with malignant course with inducible ischemia or LCA involvement should undergo surgical revascularisation.

A rare endocrine cause of electrical storm - a case report.

Sheehan's syndrome, also called Simmond's syndrome, postpartum apoplexy, postpartum pituitary necrosis, and postpartum panhypopituitary syndrome, is the name given to postpartum hypopituitarism. The syndrome is caused by an infarction in the adenohypophysis, usually precipitated by massive uterine haemorrhage and hypovolemic shock during or after childbirth. Extensive destruction of pituitary cells results in varying degree of hypopituitarism. Acute loss of adenohypophysis function can be fatal without glucocorticoid and thyroid replacement therapy and survivors will require life time hormonal replacement therapy. Most cases present in the postpartum period with lactation failure or after months to years after the delivery. In many affected women, anterior pituitary dysfunction is not diagnosed for many years. Dyselectrolytemia is one of a common presentation in Sheehan's syndrome. Herein, we report a case of a 35-year old female with a history of obstetric hysterectomy 2 years ago in view of severe postpartum haemorrhage with history of failure of lactation and three episodes of syncope since last 1½ years and now presented with polymorphic ventricular tachycardia which required DC cardioversion. She was referred as a case of long QT syndrome. On investigating further, she had hypokalaemia secondary to hypopituitarism due to Sheehan's syndrome.

Echocardiographic parameters in clinical responders to surgical pericardiectomy - A single center experience with chronic constrictive pericarditis.

BACKGROUND: Chronic constrictive pericarditis (CCP) is the end result of chronic inflammation of the pericardium. Developing countries continue to face a significant burden of CCP secondary to tuberculous pericarditis. Surgical pericardiectomy offers potential cure. However, there is paucity of echocardiography data in post-pericardiectomy patients vis-a-vis their clinical status. We studied the changes in multiple echocardiographic parameters in these patients before and after pericardiectomy. METHODS: Twenty-three patients (14 men, 9 women) who underwent pericardiectomy for CCP in the last 5 years (from January 2009 to December 2014) were subjected to detailed clinical and echocardiographic evaluation during the study period (between June 2013 and December 2014). Patients with residual symptoms of NYHA class II and below were considered as 'responders'. The data thus obtained were compared to the pre-operative parameters. RESULTS: After pericardiectomy, the incidence of vena caval congestion decreased from 100% to 15% (p<0.001). There was significant reduction in the mean left atrial size from 39.33±10.52mm to 34.45±10.08mm (p<0.001) and also the ratio of left atrium to aortic annulus from 1.93 to 1.69 (p<0.001) among 'responders' to pericardiectomy. Septal bounce was observed to persist in 5 (25%) patients after pericardiectomy. There was significant respiratory variation of 39.23±15.11% in the mitral E velocity before pericardiectomy. After pericardiectomy, this variation reduced to 14.43±7.76% (p<0.001). There was also significant reduction in the respiratory variation in tricuspid E velocities from 31.33±18.81% to 17.35±16.26% (p<0.001). After pericardiectomy, the mean ratio of mitral annular velocities, medial e': lateral e', reduced from 1.08 to 0.87 (p<0.03). The phenomenon of 'annulus reversus' was found to persist in 6 'responders', thereby reflecting a 50% reduction in its incidence after pericardiectomy (p<0.001). The ratio of mitral E to medial e' (E/e') increased from 4.21±1.35 before pericardiectomy to 6.91±2.62 after pericardiectomy (p=0.001). CONCLUSION: Among clinical responders to surgical pericardiectomy, echocardiographic assessment revealed a significant reduction in vena caval congestion, LA size, ratio of LA to aortic annulus, septal bounce, respiratory variation in mitral and tricuspid E velocities, mitral annular medial e' and the phenomenon of annulus reversus. Also, there was a significant rise in minimum tricuspid and mitral E velocities and the E/e' ratio.

A classical case of the Gasul phenomenon.

This case demonstrates the development of secondary infundibular stenosis in a 10-year-old male child with documented large non-restrictive perimembranous ventricular septal defect in infancy - the classical Gasul phenomenon.

Percutaneous tricuspid valvotomy for pacemaker lead-induced tricuspid stenosis.

Permanent pacemaker lead-induced tricuspid regurgitation is extremely uncommon. We report a patient with severe tricuspid stenosis detected 10 years after permanent single chamber pacemaker implantation in surgically corrected congenital heart disease. The loop at the level of the tricuspid valve may have caused endothelial injury and eventually led to stenosis. Percutaneous balloon valvotomy for such stenosis has not been reported from India.

Classical Cardiovascular Manifestations of Marfan Syndrome.

Presence of multiple cardiovascular manifestations of the Marfan syndrome in the same patient is not commonly encountered. We present a 49 year-old lady with this syndrome who presented with decompensated heart failure. Evaluation revealed presence of extensive Stanford type A aortic dissection alongwith severe aortic and mitral incompetence. However, the patient declined surgery and was discharged on medical management. At a year's follow-up, she had dyspnea of NYHA class II with persistent cardiovascular findings.

An extremely rare cause of Eisenmenger syndrome.

Persistent Truncus Arteriosus (PTA) is an extremely rare defect accounting for under 1% of all congenital heart disease, and over 85% of patients succumb to heart failure in infancy. We report a case of van Praagh type A3 PTA, which represents its rarest variant and accounts for only 8-10% of all patients with PTA. The presence of ductal stenosis in such cases is further exceedingly rare.

Extranodal NK/T cell lymphoma presenting as palatal perforation with oronasal fistula.

A 26 years old female presented with fever, nasal stuffiness with blood stained purulent discharge, nasal twang in voice along with non-healing palatal ulcers since three months. Examination revealed a paramidline perforation in the hard palate causing oronasal fistula formation. Histopathology and immunohistochemistry suggested a diagnosis of extranodal Natural Killer (NK)/T cell lymphoma. Multi-agent chemotherapy was instituted. We report this extremely rare case of Non-Hodgkin's Lymphoma (NHL) of the extranodal NK/T cell variety presenting as palatal perforation.