Jamal Yusuf

Phosphodiesterase-5 Inhibitor in Eisenmenger Syndrome A Preliminary Observational Study

Background— Phosphodiesterase-5 inhibitors produce a significant decrease in pulmonary vascular resistance in patients with idiopathic pulmonary arterial hypertension. We studied the effects of tadalafil, a phosphodiesterase-5 inhibitor, on short-term hemodynamics, tolerability, and efficacy over a 12-week period in patients of Eisenmenger syndrome having a pulmonary vascular pathology similar to idiopathic pulmonary arterial hypertension. Methods and Results— Sixteen symptomatic Eisenmenger syndrome patients (mean age, 25±8.9 years) were assessed hemodynamically at baseline and 90 minutes after a single dose of tadalafil (1 mg/kg body weight up to a maximum of 40 mg). The same dose was then continued daily for 12 weeks, and the patients were restudied. There was a significant decrease in mean pulmonary vascular resistance immediately (24.75±8.49 to 19.22±8.23 Woods units; P<0.005) and at 12 weeks (19.22±8.23 to 17.02±6.19 Woods units; P=0.03 versus 90 minutes). Thirteen of 16 patients (81.25%) showed a ≥20% decrease in pulmonary vascular resistance and were defined as responders. The mean systemic oxygen saturation improved significantly both immediately (84.34±5.47% to 87.39±4.34%; P<0.005) and at 12 weeks (87.39±4.34% to 89.16±3.8%; P<0.02 versus 90 minutes) without a significant change in systemic vascular resistance. None of the patients had a fall in systemic arterial pressure, worsening of systemic oxygen saturation, or any adverse reactions to the drug. The mean World Health Organization functional class improved from 2.31±0.47 to 1.25±0.44 (P<0.0001), and the 6-minute walk distance improved from 344.56±119.06 to 387.56±117.18 m (P<0.001). Conclusions— Preliminary evaluation of tadalafil has shown efficacy and safety in selected patients with Eisenmenger syndrome, warranting further investigation in this subgroup of patients.

Clinical efficacy of phosphodiesterase-5 inhibitor tadalafil in Eisenmenger syndrome--a randomized, placebo-controlled, double-blind crossover study.

OBJECTIVES In a randomized double-blind crossover trial, we compared the efficacy of phosphodiesterase-5 (PDE-5) inhibitor tadalafil with placebo in patients of Eisenmenger Syndrome (ES). The primary end point was the change in 6-minute walk test distance (6 MWD). Secondary end points were the effect of the drug on systemic oxygen saturation (SO(2) ), pulmonary vascular resistance (PVR), systemic vascular resistance (SVR), effective pulmonary blood flow (EPBF), and World Health Organization (WHO) functional class. BACKGROUND ES is a disorder with limited treatment options. Uncontrolled studies have shown PDE-5 inhibitors to be beneficial in patients of ES. METHODS Twenty-eight symptomatic adult patients of ES with weight ≥30 kg in WHO class II and III were enrolled. Patients were given 40 mg of tadalafil or matching placebo for 6 weeks followed by crossover to the other drug after a washout period of 2 weeks. Assessment of WHO class, exercise capacity by 6 MWD, and various hemodynamic parameters by cardiac catheterization was done at baseline, after 6 weeks and at the end of the study. RESULTS All patients completed the study. There was significant increase in 6 MWD following drug administration compared with baseline (404.18 ± 69.54 m vs. 357.75 ± 73.25 m, P < .001). Compared with placebo, tadalafil produced significant decrease in PVR (-7.32 ± 1.58, P < .001), resulting in significant increase in EPBF (0.12 ± 0.05, P= .03), SO(2) % (1.72 ± 0.58, P= .007), and WHO functional class (1.96 ± 0.18 vs. 2.14 ± 0.44, P= .025), with no significant change in SVR (P= NS). CONCLUSION In this first short-term placebo-controlled trial of tadalafil in patients of ES, the drug was well tolerated and significantly improved exercise capacity, functional class, SO(2) , and pulmonary hemodynamics.

Pentoxifylline Therapy for Hepatopulmonary Syndrome: A Pilot Study

H epatopulmonary syndrome (HPS) is characterized by a triad of liver disease, hypoxemia, and intrapulmonary vascular dilations (IPVDs). Its prevalence is 4% to 47% in patients with cirrhosis. Patients with HPS demonstrate a significant reduction in exercise capacity due to abnormal pulmonary circulation. Anatomic arteriovenous shunts in the lung are used during exercise and lead to exercise-induced impairment in gas exchange and exercise-induced arterial hypoxemia. The pathogenesis of HPS is unclear. Cytokinemediated injury is alleged to play a key role. Endothelin-1 and tumor necrosis factor (TNF) interaction, occurring in the lung vasculature, contribute to the development of experimental HPS. Overproduction of TNF, due to endotoxin stimulation of Kupffer cells, might be a major mechanism leading to HPS. Pentoxifylline, a nonspecific phosphodiesterase-4 inhibitor, blocks TNF synthesis and TNF-induced macrophagic nitric oxide production. Pentoxifylline prevented the development of HPS and attenuated HPS in cirrhotic rats. However, to our knowledge, pentoxifylline has not been used clinically for the treatment of HPS. In this study, we tried pentoxifylline therapy in patients with HPS.

Intracoronary fracture and embolization of a coronary angioplasty balloon catheter: Retrieval by a simple technique

We report a technique for retrieval of a balloon along with a portion of its shaft from the coronary system using a simple technique that does not involve the use of snare or any other retrieval tool. An additional angioplasty wire and a balloon catheter were used to remove the balloon from the coronary system. Cathet Cardiovasc Intervent 2003;58:473–477.

Nonsurgical Management of Cardiac Tamponade Caused by a Temporary Pacemaker Lead

Right ventricular perforation secondary to temporary pacemaker lead, resulting in cardiac tamponade, is a rare but life‐threatening complication. Surgery is usually the definitive management described for such cases. We describe successful sealing of such a perforation by percutaneous instillation of cyanoacrylate glue, through a guiding catheter in the pericardial sac at the perforation site.

Aortic rupture during aortoplasty in Takayasu arteritis – A rare complication: Case report and review of literature

Balloon angioplasty of the stenosed aorta is usually a relatively simple, yet potentially a catastrophic procedure. Aortic rupture during aortoplasty, though uncommon, carries a high mortality. We report case of a 39-year-old female with aortoarteritis with multiple arterial stenoses whose infra-renal abdominal aorta ruptured during balloon dilatation of the stent deployed in that segment. The site of aortic rupture was temporarily occluded by low-pressure inflation of the same balloon and then was sealed using a stent-graft introduced by contra-lateral femoral arterial access.

Pseudoaneurysm of the Left Ventricle in a Young Asymptomatic Female

We report a rare case of a 25‐year‐old asymptomatic female who was referred for evaluation of a cardiac murmur. She had recent history of treatment for pulmonary tuberculosis. On investigation, she was diagnosed with left ventricular pseudoaneurysm (PSA) likely secondary to rupture of myocardial tubercular abscess. She was operated successfully for the left ventricular PSA and was discharged from the hospital in a good condition. (Echocardiography 2010;27:329‐331)

Transcatheter Closure of Coronary-to-Pulmonary Fistula by Nonconventional Coils in a Patient with Tetralogy of Fallot

We report a case in which a coronary-to-pulmonary fistula was successfully occluded in a patient with tetralogy of Fallot by employing the cut floppy tips of coronary angioplasty guidewires using a new technique.

Effect of heart rate control on coagulation status in patients of rheumatic mitral stenosis with atrial fibrillation – A pilot study☆

BACKGROUND AND AIM OF STUDY Systemic thromboembolism is a major complication in patients of mitral stenosis (MS) with atrial fibrillation (AF) due to induction of hypercoagulable state. The aim was to assess the relationship, if any, between control of ventricular rate and systemic coagulation factors. METHOD 70 patients of moderate to severe MS in AF were studied. 35 patients with average heart rate > 100 beats/min over a 24 hour period assessed by Holter monitoring were considered as having a uncontrolled ventricular rate (Group A) and those with average heart rate ≤ 100 beats/min as controlled ventricular rate (Group B). 30 healthy volunteers acted as controls. RESULTS Plasma concentration of prothrombin fragment 1+2 (PF1+2) 6600 pmol/ml [interquartile range (IQR) 5400.0-9500], thrombin antithrombin III 22.0 ng/ml [IQR 18.6-28.0], and plasminogen activator inhibitor 46.8 ng/ml [IQR 44.0-54.0] were elevated in Group A as compared to Group B (5400 pmol/ml [IQR 3600-7700] p = 0.009, 16.0 ng/ml [IQR 11.0-18.5] p<0.001, and 25.8 ng/ml [IQR 20.9-34.4] p < 0.001), respectively. A significant correlation was found between heart rate and all three coagulation markers. Multivariate multiple regression analysis showed only heart rate to be an independent predictor of systemic coagulation activation and risk of thrombus formation. CONCLUSION Control of ventricular rate in subjects of MS with AF produces significant reduction in the activation of the coagulation system and may decrease risk of thrombosis.

Ruptured cardiac angiosarcoma with pulmonary metastases: a rare disease with a common (mis)diagnosis!

In Indian settings pulmonary tuberculosis remains the most common diagnosis in a patient presenting with constitutional symptoms, hemoptysis and lung opacities. We describe a case report of a fifty-year-old woman who was receiving empirical anti-tubercular drugs for a metastatic illness to lungs arising from a primary angiosarcoma in the right atrium. This rare entity was misdiagnosed and typical echocardiographic findings suggested this diagnosis.