Purendra Pati

Giant Pulmonary Artery Aneurysm With Dissection in a Case of Marfan Syndrome

![Figure][1] [![Graphic][3] ][3] A 40-year-old man presented with left-sided chest pain of 1 week in duration. He had tachycardia, tachypnea, and an early diastolic murmur with Marfanoid features. Chest x-ray showed gross cardiomegaly and a prominent main pulmonary artery (A) .

Assessment of right ventricular strain and strain rate in patients with severe mitral stenosis before and after balloon mitral valvuloplasty.

OBJECTIVE Right ventricular (RV) dysfunction in isolated severe mitral stenosis (MS) patients have prognostic significance. Study aim was to assess RV function in these subjects by strain and strain rate analysis, pre and post-balloon mitral valvuloplasty (BMV). METHODS Twenty five patients with isolated severe MS in sinus rhythm were assessed for RV function by two dimensional (2D) longitudinal strain & strain rate imaging before and after BMV and compared with that from twelve healthy age matched controls. RESULTS Patients with severe MS had significantly lower global RV systolic strain; segmental strain at basal, mid, apical septum and basal RV free wall; but similar strain at mid and apical RV free wall as compared to controls. The systolic strain rate was significantly lower only at mid septum. In addition, they had higher estimated pulmonary artery systolic pressure and RV myocardial performance index; lower tricuspid annular plane systolic excursion (TAPSE), peak systolic velocity at lateral tricuspid annulus, isovolumic acceleration and fractional area change (FAC). Global RV systolic strain as well as, segmental strain at basal, mid and apical septum showed a statistically significant rise after BMV. TAPSE and FAC also increased significantly post BMV. CONCLUSIONS RV systolic function is impaired in patients with severe MS and can be assessed by global and segmental RV strain before the appearance of clinical signs of systemic venous congestion. Impaired global and segmental RV strain values in these patients are primarily due to increased after load and improve after BMV with reduction in RV afterload.

Optimization and audit of radiation dose during percutaneous transluminal coronary angioplasty

The percutaneous transluminal coronary angioplasty (PTCA) is one of the interventional procedures which impart high radiation doses to patients compared to the other cardiologic procedures. This study intends to audit and optimize radiation dose imparted to patients undergoing PTCA. Forty-four patients who underwent PTCA involving single or multiple stent placement guided under cardiovascular X-ray machine were included in the study. Radiation doses were measured using dose area product (DAP) meter for patients undergoing single and multiple stent placements during PTCA. A dose reduction of 27-47% was achieved using copper filters and optimal exposure parameters. The mean DAP values before optimization were 66.16 and 122.68 Gy cm(2) for single and multiple stent placement respectively. These values were 48.67 and 65.44 Gy cm(2) respectively after optimization. In the present scenario, due to the increase in the number of PTCAs performed and the associated risk from radiation, periodical audit of radiation doses for interventional procedures are recommended.

Effects of aging, renal dysfunction, left ventricular systolic impairment, and weight on steady state pharmacokinetics of perhexiline.

Materials and Methods: Two hundred patients at steady-state on long-term perhexiline were identified retrospectively. The ratio of maintenance dose to steady-state plasma concentration (dose:[Px]) was correlated with the following putative determinants via simple and multiple linear regression analyses: age, weight, left ventricular ejection fraction (LVEF), and creatinine clearance (CrCl, Cockroft-Gault formula). A Mann-Whitney U test was performed to determine if severe left ventricular systolic impairment affected maintenance dose. Results: Advanced age, left ventricular systolic impairment, and renal impairment were frequently encountered. Using simple linear regression, age was a negative correlate of dose:[P] (R = 0.23, P = 0.001), whereas weight (R = 0.27, P = 0.0001) and CrCl (R = 0.30, P < 0.0001) were positive correlates. Mann-Whitney U analysis showed no difference between dose: [Px] among patients with LVEF of less than 30% versus 30% or greater. Advancing age was strongly associated with decreasing weight (R = -0.45, P < 0.00001) and calculated CrCl varied directly with weight, as expected (R = 0.66, P < 0.0001). Stepwise multiple linear regression using age, LVEF, CrCl, and weight as potential predictors of dose:[P] yielded only weight as a significant determinant. Discussion: Perhexiline has become a “last-line” agent for refractory angina as a result of complex pharmacokinetics and potential toxicity. Use has increased predictably in the aged and infirm who have exhausted standard medical and surgical therapeutic options. Beyond genotype, the effect of patient characteristics on maintenance dose has not been explored in detail. In this study, dose requirement declined with age in a frail and wasting population as a result of weight-related pharmacokinetic factors. LVEF had no apparent effect on maintenance dose and should not be considered a contraindication to use. Conclusion: A weight-adjusted starting dose may facilitate the safe and effective prescription of perhexiline and is calculated by 50 + 2 × weight (kg) mg/d, rounded to the closest 50 mg/day.

Audit of radiation dose during balloon mitral valvuloplasty procedure

Radiation doses to patients during cardiological procedures are of concern in the present day scenario. This study was intended to audit the radiation dose imparted to patients during the balloon mitral valvuloplasty (BMV) procedure. Thirty seven patients who underwent the BMV procedure performed using two dedicated cardiovascular machines were included in the study. The radiation doses imparted to patients were measured using a dose area product (DAP) meter. The mean DAP value for patients who underwent the BMV procedure from one machine was 19.16 Gy cm(2) and from the other was 21.19 Gy cm(2). Optimisation of exposure parameters and radiation doses was possible for one machine with the use of appropriate copper filters and optimised exposure parameters, and the mean DAP value after optimisation was 9.36 Gy cm(2).

Feasibility of Angioplasty and Stenting for Abdominal Aortic Lesions Adjacent to Previously Stented Visceral Artery Lesions in Patients with Takayasu Arteritis

Two young female patients with Takayasu arteritis presented with symptomatic long-segment abdominal aortic stenosis in the vicinity of previously deployed celiac and renal artery stents that projected markedly into the narrowed aortic lumen. Crushing or distortion of the visceral artery stents during aortic angioplasty was avoided by performing simultaneous or alternating balloon dilatations in the aorta and in the visceral artery stents. Consequently, the visceral artery stents remained patent and shortened longitudinally, allowing unhindered deployment of Wallstents in the adjacent aorta and abolition of a pressure gradient across the aortic lesions. Access to side branches covered by the Wallstent was obtained without difficulty, enabling the performance of balloon dilatation in multiple side branches and ostial stent deployment in a renal artery. These techniques could increase the scope of endovascular therapy in the treatment of patients with Takayasu arteritis.

Isolation of left subclavian artery with reversal of neurological and hemodynamic abnormalities after percutaneous closure

Isolation of left subclavian artery is a rare congenital anomaly. In this abnormality, the left subclavian artery arises from the homo-lateral pulmonary artery rather than from aorta. This condition is often diagnosed by angiography and treated by surgery. The authors present a case, which had vertebro-basilar insufficiency, subclavian steal phenomenon and pulmonary plethora. All these clinical signs disappeared by a simple percutaneous intervention.

The Shadow Within: A Colossal Left Atrium

A 32-year-old male, who was diagnosed with rheumatic heart disease 9 years prior, presented with progressively worsening exertional dyspnea and palpitations for 2 years. Clinical examination revealed atrial fibrillation with features of severe mitral regurgitation (MR). His chest x-ray (in posteroanterior view) exposed an enormously enlarged cardiac silhouette with a cardiothoracic ratio of 0.88. There was splaying of the carinal angle and distinct double shadows along the right heart border indicative of left an enlarged atrium (LA) (Fig. 1A). Transthoracic echocardiogram (Philips iE33, Philips Medical Systems, Andover, MA, USA) disclosed a thick calcified rheumatic mitral valve together with severe MR and mild aortic regurgitation. As presumed from the x-ray projections, the LA was hugely distended measuring 15.2 9 12.8 cm (Fig. 1B and movie clip S1). Cardiac magnetic resonance imaging (1.5 Tesla MRI scanner, GE Medical Systems, Milwaukee, WI, USA) was also performed which demonstrated the LA compressing all other cardiac chambers with a calculated volume of 1950 mL (Fig. 2). The patient has been advised valve replacement and is presently awaiting surgery. The LA is ordinarily located in the posterior aspect of the mediastinum and does not contribute to any cardiac border on a posteroanterior view of the normal chest roentgenogram. However, abnormal enlargement of the LA proceeds in a rightward direction and may even about the right chest wall, mimicking a pleural effusion. A giant LA is defined by a diameter larger than 8 cm in the transthoracic echocardiogram in parasternal long-axis view, and is almost always due to rheumatic mitral valve disease. The plausible etiology is postulated to be pancarditis of rheumatic origin involving the left atrial wall. The left atrial size depicted in this case appears to

A Bird in Hand or Two in the Bush

A 37-year-old man presented with a 6-year history of recurrent paroxysmal palpitations. The episodes occasionally were accompanied by presyncope and terminated by intravenous verapamil. Symptoms persisted despite amiodarone therapy. The patient underwent electrophysiology (EP) study. Baseline ECG (Fig. 1A) showed preexcitation. Decremental atrial stimulation via the coronary sinus revealed the pattern shown in Figure 1B. What is the diagnosis?