Loeffler endocarditis: silent right ventricular myocardium!

We present the case of a 54-year-old male patient with Loeffler endocarditis. It is a rare disorder characterized by fibrous thickening of the endocardium leading to apical obliteration and restrictive cardiomyopathy resulting in heart failure, thromboembolic events or atrial fibrillation. To the best of our knowledge, this is the first case reporting the electrical silence of the right ventricular (RV) apex caused by fibrothrombotic thickening of this area. Under these circumstances RV apical implantation of an implantable cardioverter defibrillator (ICD) or pacemaker electrode may lead to unsuccessful stimulation of these devices.

Diabetes mellitus and female gender are the strongest predictors of poor collateral vessel development in patients with severe coronary artery stenosis.

BACKGROUND: Coronary collateral vessel development (CVD), i.e., arteriogenesis, is regarded as one of the most important mechanisms­along with angiogenesis­to result in protection of the myocardium. Coronary CVD is associated with a reduction in infarct size, future cardiovascular events and improved survival in patients with occlusive coronary artery disease by enhancing regional perfusion in the chronically ischemic myocardium. In the present study, we aimed to investigate the relation of cardiovascular risk factors and hematological parameters with collateral development in patients with severely stenotic (≥95%) and totally occluded coronary artery disease including at least one major coronary artery. MATERIALS AND METHODS: The study population was selected from the patients who underwent coronary angiography between January 2008 and March 2009. Five hundred and two patients who had at least one coronary artery stenosis ≥95% (368 men; mean age 59 ± 10 years) comprised the study population. Of the 502 patients, 228 had total occlusion in at least one major epicardial coronary artery. Collateral artery grading was performed by using Cohen-Rentrop method to the vessel with coronary artery stenosis of ≥95% and patients with chronic total occlusions (CTO). Patients with grade 0-1 collateral development were regarded as the poor collateral group, and patients with grade 2-3 collateral development were regarded as the good collateral group. RESULTS: Two hundred and fifty-eight (51%) of 502 patients had poor collateral development, and 244 (49%) had good collateral development. Logistic regression analysis revealed that DM was independently associated with poor CVD in patients with ≥95% stenosis (p < 0.001). Additionally, female gender and DM were found to be independently associated with poor CVD in patients with CTO (p = 0.005 and p < 0.001, respectively). Monocyte count was found to be independent of CVD neither in patients with ≥95% stenosis nor in patients with CTO. CONCLUSION: Our data show that DM is an independent factor for poor coronary CVD both in patients with severe coronary artery stenosis and in patients with CTO. Female gender or being in post-menopausal period is another negative risk factor for poor CVD in addition to DM in patients with CTO.

Absence of posterior mitral leaflet with secundum atrial septal defect.

A rare case of a 54-year-old woman with absence of congenital posterior mitral leaflet, moderate mitral insufficiency, and large secundum-type atrial septal defect is reported. Two-dimensional color Doppler and transesophageal echocardiography revealed complete absence of the posterior mitral leaflet, a thick muscular formation replacing the posterior leaflet, a 3.3-cm secundum type atrial septal defect, and severe pulmonary hypertension. This report describes the rare case of congenital absence of posterior mitral leaflet associated with secundum type large atrial septal defect in a middle-age woman.

Hypertrophic obstructive cardiomyopathy causing severe right and left ventricular outflow tract obstruction.

An 18-year-old male patient presented with a 3-year history of exertional dyspnea, dizziness, and angina. Echocardiography showed advanced hypertrophy of the left ventricle (LV), right ventricle (RV) free wall, and interventricular septum. There were apparent muscular bundles especially at the level of the right ventricular outflow tract (RVOT). Maximal pressure gradients across the RVOT and left ventricular outflow tract (LVOT) were 141 mmHg and 66 mmHg, respectively. There was also grade 2 aortic regurgitation. Transesophageal echocardiography and cardiac magnetic resonance imaging confirmed these findings. Despite treatment with propranolol and cibenzoline, the patient remained symptomatic with unchanged pressure gradients. Corrective surgery including an extensive muscular resection of the RVOT, minimal resection of the LVOT, and interposition of a graft patch in the RVOT resulted in complete disappearance of the RVOT gradient and a significant decrease to 28 mmHg in the LVOT gradient. During a year follow-up, aortic valvular insufficiency remained clinically stable and the patient was asymptomatic. This is the first case of hypertrophic obstructive cardiomyopathy with predominant RVOT obstruction treated by myectomy and patch graft interpositioning.

Cardiac findings in Behçet's patients.

Behçet's disease is a chronic multi-system inflammatory disorder and the severity and clinical manifestations of Behçet's patients may show geographic variation. We aimed to detect the cardiac findings in 30 Behçet's patients and compare them with the normal population (n = 29). We used color-doppler echocardiography and transesophageal echocardiography in combination. We calculated manually QT intervals and QT dispersion (QTd) from twelve-lead ECG recordings. There was no E/A inversion and coronary ischemia in all patients or control group. The E velocity difference between groups was not significant. The mean A velocity was significantly lower in Behçet's patients than normal group. The mean DT was 154.4 +/- 5.8 msec in Behçet's patients and 122.59 +/- 0.96 msec in control group (P < 0.0001). The mean IVRT was 75.66 +/- 1.36 msec in Behçet's patients and 69.1 +/- 0.55 msec in control group (P < 0.0001). There was no QTc time difference between the Behçet's patients and the control group. The mean QT dispersion (QTd) interval was 45.46 +/- 2.65 msec in Behçet's patients and 31.83 +/- 1.23 msec in control group (P < 0.0001). Atrial septal aneurysm, mitral valve prolapse and insufficiency, tricuspid valve insufficieny, and pulmonary hypertension frequencies in Behçet's patients were significantly higher than in the control group. We concluded that Behçet's cardiac involvement may effect cardiac structure and cause diastolic dysfunction, electrical instability and structural abnormalities. We also concluded that cardiac involvement in Behçet's disease may be specific for this geographic area.

Persistent atrial standstill and idioventricular rhythm in a patient with thalassemia intermedia.

We present a 57-year old male patient with thalassemia intermedia and right heart failure. He had a 30-year history of anemia and short-term iron therapy without blood transfusion. Hemoglobin level was 7.1 g/dl and hematocrit was 22.7%. White blood-cell and platelet counts, and serum ferritin level were normal. Electrocardiography showed irregular narrow QRS bradyarrhythmia, suggesting slow atrial fibrillation at a mean rate of 35 beats/min. Echocardiographic examination revealed dilatation of the right atrium and ventricle, depressed systolic right ventricular function, advanced tricuspid regurgitation, and mild pericardial effusion. In the electrophysiologic study, no electrical activity was recorded in the right atrium. It was inexcitable at multiple sites and no retrograde conduction to the right atrium could be elicited by ventricular pacing. His bundle (HB) recording showed fixed retrograde HB activation with ventricular rhythm originating from different foci. Retrograde V-H conduction time during ventricular rhythm was 95 msec and did not change. There was no retrograde nodal conduction. A VVIR pacemaker was implanted. During a six-month follow-up, he felt well, his functional capacity was NYHA class II, and his basic rhythm was widened QRS arrhythmia with a rate of 20 beats/min. To the best of our knowledge, atrial electrical inactivity together with right-heart failure and pericarditis confined to the right heart chambers has hitherto not been reported in thalassemic disorders.

Reperfusion arrhythmias: are they only a marker of epicardial reperfusion or continuing myocardial ischemia after acute myocardial infarction?

Reperfusion arrhythmias are associated with epicardial reperfusion but may also be a sign of vascular reperfusion injury which can be seen as no-reflow phenomenon on coronary angiography and predicts in-hospital complications and recovery of left ventricular (LV) function. No-reflow phenomenon (thrombolysis in myocardial infarction [TIMI]

High-pressure pulmonary artery aneurysm and unilateral pulmonary artery agenesis in an adult.

The presence of a pulmonary artery aneurysm, major aortopulmonary and coronary-pulmonary collateral vessels, and severe pulmonary hypertension in an adult with unilateral pulmonary artery agenesis and previous patent ductus arteriosus ligation is very rare. A 34-year-old man experienced these conditions. When he was 10 years old, catheterization and angiography revealed right pulmonary artery agenesis, dilation of the main pulmonary artery, multiple collateral vessels extending from the aorta to the right pulmonary system, and a patent ductus arteriosus (shunt ratio, 3.57) that was then ligated; the other conditions were not corrected. This adult patient was in New York Heart Association functional class II; mild central cyanosis was detected only during exercise. The right pulmonary arterial system was seen only at the right hilar area via collateral vessels from the subclavian, bronchial, internal mammary, and intercostal arteries. Angiography revealed collateral vessels from the right and circumflex coronary arteries to the right pulmonary system. The right intraparenchymal pulmonary arterial systems were patent but of small diameter (pulmonary artery pressure, 85 mmHg; ratio of peak right-to-left ventricular pressure, 0.94; peak pulmonary pressure unresponsive to 100% oxygen). Pulmonary vascular resistance was not estimated because of the risk of aneurysmal rupture. We concluded that irreversible pulmonary hypertension had developed (delayed by the patent ductus arteriosus ligation in childhood) and that the patient's only chance for survival was heart-lung transplantation. To sustain the patient until surgery, we administered sildenafil. Herein, we describe the vascular conditions that accompany unilateral absence of the pulmonary artery, and therapeutic methods.

Treatment of an iatrogenic left internal mammary artery to pulmonary artery fistula with a bovine pericardium covered stent.

We report a case with an acquired fistula between the left internal mammary artery and the pulmonary artery following coronary bypass surgery treated with a bovine pericardium covered stent. We also reviewed similar cases reported previously.

Atrioventricular nodal reentrant tachycardia with paroxysmal atrial fibrillation: clinical and electrophysiological features and predictors of atrial fibrillation recurrence following elimination of atrioventricular nodal reentrant tachycardia.

INTRODUCTION: Clinical and electrophysiological characteristics of patients with atrioventricular nodal reentrant tachycardia (AVNRT) and paroxysmal atrial fibrillation (AF) have not been studied in a large patient cohort. We aimed to define the clinical features and cardiac electrophysiological characteristics of these patients, and to examine the incidence and identify predictors of AF recurrences after elimination of AVNRT. METHODS AND RESULTS: Thirty-six patients with AVNRT and documented paroxysmal AF (Group 1) and 497 patients with AVNRT alone undergoing ablation in the same period (Group 2) were studied. There were no significant differences between groups regarding clinical features, except age, which was higher in Group 1 (p<0.001). Presence of atrial vulnerability (induction of AF lasting>30 seconds) and multiple AH jumps (>or=50 ms) before ablation were significantly more prevalent in Group 1 (p<0.001, p=0.010 respectively). During follow-up of 34 +/- 11 months, AF recurred in 10 patients (28%) in Group 1, while 2 patients in Group 2 (0.4%) developed paroxysmal AF (p<0.001). Univariate predictors of AF were: left atrial diameter>40 mm (p=0.001), presence of mitral or aortic calcification (p=0.003), atrial vulnerability after ablation (p=0.015) and valvular disease (p=0.042). However, independent predictors of AF recurrences were left atrial diameter>40 mm (p=0.002) and the presence of atrial vulnerability after ablation (p=0.034). CONCLUSION: In patients with both AVNRT and paroxysmal AF, the recurrence rate of AF after elimination of AVNRT is 28%. Left atrial diameter greater than 40 mm and atrial vulnerability after elimination of AVNRT are independent predictors of AF recurrences in the long term.

Two female nonsmoker Buerger's disease cases with anticardiolipin autoantibodies and a poor prognosis.

We present two female nonsmoker Buerger's disease cases with anticardiolipin autoantibodies and a poor prognosis. One was a 64-year-old female who has had multiple lower and upper extremity amputations, while the other was a 32-year-old female with extremity and visceral artery involvement. Since both were positive for anticardiolipin antibodies, we speculate that Buerger's disease is an autoimmune disorder.

Increased myocardial ischemia during nitrate therapy: caused by multiple coronary artery-left ventricle fistulae?

We present the case of a 54-year-old man who had crescendo angina during nitrate therapy. Selective coronary angiograms showed no atherosclerotic lesions, but did show plexuses of intramural vessels that connected the distal thirds of the left and right coronary systems with the left ventricle. The cause of our patient's increased myocardial ischemia during nitrate therapy may have been the coronary "steal" phenomenon.

Left ventricular apical aneurysm as a consequence of diffuse type congenital nonfamilial supravalvular aortic stenosis in a 30-year-old female.

Congenital nonfamilial supravalvular aortic stenosis (SVAS) is relatively rare, its diffuse type being the least common. We present a 30-year-old woman with diffuse SVAS complicated with left ventricular apical aneurysm. We believe that subtle left ventricular myocardial ischemia or infarction and long-lasting severe pressure overload to the apical chamber caused LV apical aneurysm in our case. Acquired LV apical aneurysm secondary to supravalvular aortic stenosis, in the absence of atherosclerotic coronary artery disease and hypertrophic obstructive cardiomyopathy, has not been described before.

Clinical and hemodynamic follow-up of a patient after operation for dissection of an ascending aortic aneurysm secondary to coarctation of the aorta.

We present clinical follow-up of a 20-year-old male with an aortic aneurysm secondary to aortic coarctation. The diagnosis of aortic aneurysm secondary to aortic coarctation was made in 1997. The patient did not agree to undergo any invasive or therapeutic procedures at that time. He presented to an emergency unit with severe chest pain after chest trauma obtained during judo exercises in 1998. Two-dimensional echocardiography showed bicuspid aortic valves, an ascending aortic aneurysm 6 cm in diameter with an intimal flap and false lumen, aortic coarctation distal to the left subclavian artery, and aortic insufficiency secondary to annular dilatation. Type II aortic dissection was confirmed by transesophageal echocardiography, which showed the dissection was confined to the ascending aorta. The dissection extended to the beginning of the arcus aorta. Following stabilization of the patient's clinical condition, balloon coarctation angioplasty was performed to reduce afterload and hypertension and to facilitate femoral artery cannulation for cardiopulmonary bypass. Surgical procedures included resection of the aortic valve and prosthetic valve implantation, resection of the ascending aorta, and interposition of a 22 mm Hamashied tubular vascular graft. At a follow-up visit 6 years later, the patient reported being easily fatigued and having palpitations. He had been suffering from hemolytic anemia and mild renal function impairment. Cardiac catheterisation and angiography showed a 40 mmHg gradient due to kinking of the aortic graft and no gradient at the coarctation site. We postulated the kinking of the aortic vascular graft may be related to an inappropriate vascular graft length. We also thought that the severe hemolysis was attributable to the disturbance of blood flow by a jet of blood at the site of the kinking aortic vascular graft. A second operation was performed because the renal function of the patient had decreased progressively and hemolysis symptoms increased. After the second operation, hemolysis on peripheral blood smears had disappeared and renal function had shown progressive improvements.

Chylous ascites and pleural effusion secondary to constrictive pericarditis presenting with signs of lymphatic obstruction.

Chylous ascites is a clinical entity characterized by accumulation of milky fluid containing high amounts of triglycerides in the peritoneal cavity. The cause is usually lymphatic obstruction secondary to neoplastic processes. Constrictive pericarditis rarely causes cylous ascites through elevated venous pressure and lymphatic stasis. To the best of our knowledge, there is no report of constrictive pericarditis leading to chylous ascites in a patient presenting with objective lymphangiographic findings of lymphatic obstruction rather than stasis. We present a case of chylous ascites and pleural effusion secondary to constrictive pericarditis presenting with signs of lymphatic obstruction in lymphangio-graphy, in whom complete clinical and laboratory improvement was achieved after pericardiectomy.

Effects of afterload increase on systolic and diastolic functions of the myocardium after myocardial infarction.

The evaluation of noninfarcted zone function after myocardial infarction by the use of noninvasive methods is very important. The authors speculated that phenylephrine, which increases systemic vascular resistance and blood pressure and has no effect on central ischemic and border-zone myocardium but does have an effect on remote myocardium, could be used as a stress agent as information is gathered about the functional capacity of the left ventricle and the status of coronary arteries in patients with recent myocardial infarction. Forty-six patients with recent myocardial infarction (5 women, 41 men; mean age: 53.6 +/-9.3 years) and 15 individuals with normal findings from coronary angiography and ventriculography (9 women and 6 men; mean age: 39.0 +/-11.2 years) were included in the study. The study was performed on the 4th or 5th day of the myocardial infarction. Preejection period/left ventricular ejection time (PEP/LVET), diastolic mitral flow velocity, isovolumic relaxation time (IVRT), and deceleration time (DT), were measured before and after the phenylephrine infusion, with M-mode, pulse wave, and continuous-wave echocardiography. After pressor stress with phenylephrine infusion, all the parameters were measured again. Coronary angiography and ventriculography were performed on all the patients on the 7th to 10th day of the myocardial infarction. All the patients were grouped according to their ejection fraction and the number of involved coronary arteries. The increase in the PEP/LVET ratio in Group 1 (left ventricle ejection fraction [EF] below 40%) and multivessel coronary artery lesion group was significant (p<0.01). PEP/LVET ratio decreased significantly in both Group C (patients with normal-appearing coronary arteries and ventriculographies) and the single-vessel coronary disease group. Although the early diastole flow/atrial systole flow (E/A) ratio increased significantly in the 3 groups, the 0.5 and more increase in E/A ratio had high sensitivity (86%) and specificity (80%) in differentiating the low EF group. The 0.5 and more increase in E/A ratio had 65% sensitivity and 69% specificity in differentiating the multivessel coronary stenosis group. A deceleration time of 130 msec and below in basal conditions had a high sensitivity (86%) and specificity (92%) for detecting the group in which EF was below 40%. After phenylephrine infusion, the shortening of IVRT was significant in Group 1 (p<0.01). Phenylephrine, which has been shown to be an alpha-1 receptor agonist in low doses and effective only on remote myocardial function, may be given with low complication rates in the early postinfarction period. The increase in PEP/LVET ratio, 0.5 and more increase in E/A ratio, and shortening of DT and IVRT after phenylephrine infusion may be indicators of low LV functional capacity and widespread coronary artery disease. This test may suggest performance of early invasive detection of coronary artery disease and early revascularization. This study may also be interesting from a pathophysiological point of view.

Prospective evaluation of von Willebrand factor release after multiple and single stenting.

The hypothesis that von Willebrand factor (vWf) release after multiple coronary stenting may be higher than release after single coronary stenting was tested. Preliminary data suggest that multiple stenting is a predictor of thrombosis, and vWf levels in the coronary sinus reflect coronary endothelial injury. Therefore vWf as an indicator of thrombogenesis and endothelial injury was studied. vWf levels were obtained in the coronary sinus at the prestenting and poststenting period in 50 patients with ischemic heart disease who underwent elective coronary stenting (25 patients in single stent group and 25 patients in multiple stent group). Eight subjects who underwent diagnostic coronary angiography were used as controls. vWf levels increased significantly after multiple stenting and vWf levels were significantly different from vWf levels after single stenting. In single stent group, vWf levels in type C lesions were significantly different from levels detected in type A and B lesions. Multiple coronary stenting induces a rapid increase in vWf expression in the coronary circulation. These changes may contribute to the pathogenesis of acute or subacute stent thrombosis and restenosis after multiple stenting.

The importance of von Willebrand factor level and heart rate changes in acute coronary syndromes: a comparison with chronic ischemic conditions.

The pathogenesis of acute coronary syndrome (ACS) and transient myocardial ischemia (TMI) is not completely understood. Therefore, the authors studied the biological indicators of thrombogenesis and sympathetic activity. The study was conducted on 50 patients with acute coronary syndrome and 30 patients with stable angina pectoris. Treatment was standardized with low-molecular-weight heparin and 300 mg aspirin/day but with no IIb/IIIa inhibitors, an oral beta-blocker, diltiazem 60 mg tid, glyceryl trinitrate i.v. in patients with ACS but with mononitrates orally and low-molecular-weight heparin in patients with stable angina. Twenty-four-hour continuous ECG monitoring and ST segment analysis were performed on day 2 of admission and heart rate analysis was performed 10, 5, and 1 minute before and during the myocardial ischemia periods. Blood sampling for von Willebrand factor (vWf) determination was performed through a peripheral vein at 8 AM, noon, 6 PM and 10 PM and half an hour after the description of angina. The patients with ACS were grouped as transient myocardial ischemia positive (n = 20) and negative (n = 30). The patients with stable angina were designated as the control group (n = 30). The detected vWf levels at 4 different daytime periods in patients with ACS were significantly higher than those in patients with stable angina. At the 6 PM to 10 PM period, the vWf level increase was significantly higher in patients with TMI than in the patients without TMI. At the 8 AM to noon period, the detected vWf levels decreased significantly in both TMI groups. During the nocturnal ischemia periods, the increase in vWf levels immediately after angina was significantly more apparent than the detected changes during daytime ischemia. Analysis showed that heart rates before the ischemia during stable angina episodes were significantly higher than those in TMI (-) (silent) angina. The heart rate difference between 10 minutes before and during the ischemia in the angina group was significantly different from that during TMI (-) (silent) ischemia. The heart rates at the times related to ischemia in the nocturnal period were significantly lower than those in the daytime period. The heart rate differences between the ischemia-related times and during the ischemia were significantly higher in daytime ischemic attacks than in nocturnal ischemic attacks. The study confirms that the vWf level, which is an indicator of thrombogenesis, was significantly increased in patients with ACS. Nocturnal ischemia is associated with thrombogenesis. Daytime ischemia is associated with increased sympathetic activity, and symptomatic ischemia is usually associated with increased sympathetic activity.