Girish R. Sabnis

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.

Idiopathic pseudoaneurysm of mitral–aortic intervalvular fibrosa with rupture into the left atrium

A 30-year-old male presented with insidious onset of dyspnoea of NYHA class II and frequent palpitations over 6 months. Examination revealed cardiomegaly with hyperdynamic apex and grade 4/6 apical pansystolic murmur. Transthoracic echocardiography in a parasternal long axis demonstrated a large pulsatile echo-free aneurysmal sac from the distal left ventricular outflow tract (LVOT; Panel A and see Supplementary data online, Videos S1 and S2 ). Transoesophageal imaging profiled the sac with an 8-mm wide neck at the LVOT, close to the aortic valve and a 10-mm wide communication with the left atrium (LA; Panel B ). This led to a large, …

Validation of ‘left ventricular early inflow-outflow index’: A novel echocardiographic method for quantification of mitral regurgitation in an Indian population with special focus on rheumatic etiology

Background Quantification of mitral regurgitation (MR) has always required an “integrated approach” as there is no single gold-standard method. We investigated a new Doppler-derived parameter “left ventricular early inflow-outflow index (LVEIO)” for the quantification of MR and its likelihood to predict severe MR in correlation with already established parameters in an Indian population including a large subset of patients with rheumatic etiology. Methods A prospective study was performed at a major tertiary care center in western India over a 5-month period. Five hundred patients diagnosed with isolated MR including 260 (52%) patients with rheumatic etiology were included in the study after applying exclusion criteria. We analyzed MR using color flow jet, effective regurgitant orifice area (EROA), and vena contracta (VC) width. LVEIO is a simplification of the regurgitant volume (RV) method, which was calculated as “E velocity divided by LV outflow velocity integrated over the systolic ejection period left ventricular outflow tract velocity time integral” and compared with the established parameters. Results LVEIO was 4.65 ± 1.45, 6.56 ± 1.52, and 9.91 ± 3.70 among patients diagnosed with mild, moderate, and severe MR, respectively (p < 0.001). Those with LVEIO ≥8 were the most likely to have severe MR (positive likelihood ratio: 10.42). LVEIO had specificity of 93.25% for diagnosis of severe MR with positive predictive value of 86.36%. There was positive correlation observed between LVEIO and VC width (r = 0.591), RV (r = 0.410), and EROA (r = 0.778) (all p < 0.001) in the Pearson correlation test. The specificity of LVEIO remained consistent in diagnosing severe MR in patients with rheumatic etiology. Conclusion LVEIO is a simple yet specific Doppler echocardiographic parameter for estimation of severity of MR including that of rheumatic etiology.

Catastrophic Sudden Cardiac Death. Are there Lessons to be Learnt

Background: Sudden cardiac death is a catastrophic event which leads to loss of young and middle-aged population with dire consequences. Sometimes, a careful history, physical examination and most importantly electrocardiogram (ECG) can help in suspecting the common causes in young population like underlying channelopathies and cardiomyopathies which can further be diagnosed using echocardiography, cardiac MRI and in some cases genetic tests. Case summary: A 33-year-old male presented with sycope and ventricular tachycardia leading to death. This patient was diagnosed to have Arrythmogenic right ventricular dysplasia (ARVD) on post mortem pathological examination with features of fatty infiltration and thinning of right ventricular myocardium. Furthermore, on histologically fatty infiltration with inflammatory infiltrates were visualized. Discussion: Clinical awareness amongst physicians about cardiomyopathies especially ARVD with its subtle yet suggestive ECG changes is the need of the hour. ARVD is a rare disease and can be diagnosed with certainty by analyzing ECG and adding imaging to it. When diagnosed cases are treated appropriately, at least a few cases of sudden cardiac death can be averted.

Cardio-Renal Syndrome

Although newer pharmacologic and interventional therapies for the treatment of Heart failure continue to evolve, the optimum strategy of management for the patients with cardio renal syndrome is still unanswered. Various pathophysiologic mechanisms have been proposed including the low output hypothesis and the raised renal venous pressure hypothesis. These mechanisms have been tested and challenged in many trials. Although, modest benefit has been achieved with management strategies targeting these hypotheses. Role of biomolecules and neurohormones such as BNP, adenosine has provided newer insights in mechanisms of Cardio-renal syndrome (CRS). However, when studied in clinical trials, drugs targeting these pathways did not have significant clinical outcomes. Diuretics remain cornerstone of management. But their propensity to cause AKI and electrolyte disturbances when used at higher dosages limits their use. Vasodilator proves of clinical benefit in raised CVP states. But risk of hypotension and constant hemodynamic monitoring preclude their use in many clinical situations. Ultrafiltration had modest success in the initial studies, but recent trials have questioned their role in patients with ADHF. Pharmacologic therapies such as nesiritide, adenosine antagonists and vasopressin antagonists are still in trial phases and benefit has not yet been proved. Thus, our knowledge of CRS still needs depth and understanding of pathophysiologic mechanisms which when achieved may result in improved clinical outcomes and patient care. This review article on focuses on pathophysiology and management strategies of patients with CRS.

A rare and treatable cause of hypertension in pregnancy

Dear Editor A 25yearold pregnant patient in the 24th gestational week was referred for evaluation of resistant hypertension. There was significant obstetric history of one premature delivery with neonatal death and two instances of intrauterine fetal loss. The patient was asymptomatic except for occasional headaches. Hypertension, diagnosed during the first gestation 4 years ago, was poorly controlled in spite of the use of multiple antihypertensive agents. Clinical examination revealed a significant blood pressure difference in the upper and lower extremities (upper limb 178/106 mm Hg vs lower limb 120/94 mm Hg) with weak lower limb pulses. As part of the standard workup for secondary hypertension, echocardiography with color Doppler was performed, which revealed concentric left ventricular hypertrophy with good biventricular function. Interrogation of the abdominal aorta revealed marked narrowing with turbulent flow (Figure A and B). Levels of Creactive protein and erythrocyte sedimentation rate were both within the normal range. A provisional diagnosis of aortoarteritis involving the abdominal aorta was made—the socalled middle aortic syndrome.1 Cardiac catheterization with aortography was performed at the 28th gestational week with adequate measures to minimize fetal radiation exposure, confirming the presence of long segment narrowing of the abdominal aorta (Figure C) with pressure gradients of 86 mm Hg. A selfexpandable stent of 14 mm×70 mm (Epic, Boston Scientific) was deployed at the site of maximum narrowing (Figure D). Immediately following the procedure, the aortic pressure gradient decreased by more than half to 40 mm Hg. Thereafter, hypertension could be adequately controlled with monotherapy and the patient had an uneventful caesarean section at term with good outcome. To our knowledge, this is the first case describing interventional therapy for middle aortic syndrome during pregnancy with excellent fetomaternal outcome. Middle aortic syndrome may be secondary either to a congenital anomaly or acquired conditions such as Takayasu arteritis, infection, obliterative panarteritis, neurofibromatosis, retroperitoneal fibrosis, fibromuscular dysplasia, and mucopolysaccharidosis.2 Changes in the aortic wall during pregnancy, coarctationassociated aortopathy, and longstanding hypertension combine to increase the risk of aortic rupture/dissection. Also, poorly controlled hypertension leads to adverse fetal (growth retardation, placental abruption, and premature delivery) and maternal (renal failure and hypertensive crisis) outcomes.3 Given the unsatisfactory response to multiple antihypertensive agents and their adverse effect, intervention with stenting is preferred over a conservative approach.4 The procedure was carried out during the 28th week of gestation to minimize the effect of radiation on fetal organogenesis as well as the risk of preterm labor. The procedure is generally associated with low risk and subsequently enables adequate blood pressure control with good pregnancy outcome.

A rare endocrine cause of electrical storm - a case report

Abstract 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.

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 balloon valvuloplasty with inoue balloon catheter technique for pulmonary valve stenosis in adolescents and adults

Background Percutaneous balloon pulmonary valvuloplasty is the procedure of choice for uncomplicated severe or symptomatic pulmonary stenosis. The present study describes our experience in balloon pulmonary valvuloplasty using the Inoue balloon catheter in adolescent and adult patients. Aims To assess the immediate and mid-term outcomes of percutaneous balloon valvuloplasty with Inoue balloon catheter in adolescent and adult patients. Methods and results Between June 2010 and July 2015, we performed percutaneous balloon pulmonary valvuloplasty with Inoue balloon catheter in 32 patients (59.37% females) aged 8 to 54 years (mean 23.6 ± 11.5). Following the procedure, the mean right ventricular systolic pressure and the pulmonary valvular peak-to-peak systolic gradient decreased from (121.6 ± 42.4 to 61.19 ± 24.5 mmHg, p = 0.001) and (100.9 ± 43.3 to 36.4 ± 22.5 mmHg, p = 0.001), respectively. Twenty patients (Group A) showed immediate optimal results with post-procedure peak systolic gradient <36 mmHg while 12 patients (Group B) had suboptimal results. An increase in pulmonary regurgitation by one grade was detected in 17 patients (53.2%). Twenty-three patients available for follow-up (mean duration, 2.75 years [range 0.25–5 years]) had a mean residual peak gradient of 23.6 ± 2.51 mmHg on Doppler echocardiography with attenuation of reactive RVOT stenosis in all Group B patients. There was no further increase in grade of pulmonary regurgitation or restenosis on mid-term follow-up. Conclusion Percutaneous Inoue balloon technique is an attractive alternative with excellent mid-term results for adolescents and adults with isolated pulmonary stenosis.

Classic cor triatriatum with opposing shunts at the atrial level: a rare association

A 6-year-old girl presented with progressive dyspnoea and cyanosis. Examination revealed grade 1 clubbing, cyanosis (saturation of 85%), right ventricular (RV) heave, and loud S2. ECG and chest radiograph showed evidence of RV pressure overload (Supplementary material online, Figures S1 and S2 ). Transthoracic echocardiography demonstrated a membrane dividing the left atrium (LA) into a proximal accessory chamber (AC) and a distal main chamber, consistent with classic cor triatriatum ( Panel A — parasternal long …