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Hepatopulmonary syndrome and right ventricular diastolic functions: an echocardiographic examination.

AIM: Liver functions are affected in the course of cardiac diseases, and similarly, liver diseases affect cardiac functions. Many studies in the literature have shown that left ventricular systolic and/or diastolic dysfunction may develop during chronic liver disease. However, there are limited studies investigating right ventricular functions during chronic liver diseases. METHODS: A total of 84 patients who had no systolic and/or diastolic dysfunction in the left ventricle (LV) were evaluated; 46 patients with liver cirrhosis; 10 (21.74%) cirrhotic patients with hepatopulmonary syndrome (HPS) (group 1), 36 (78.26) cirrhotic patients without HPS (group 2), and 38 healthy individuals were treated as control. RESULTS: Right ventricular diastolic dysfunction was determined in all patients of group 1 (100%), 26 of group 2 (72.22 %), and 4 of the controls (10.52%) (P<0.05). Tricuspid deceleration time (dt) was significantly different between the groups (P<0.05). In addition, right atrium (RA) diameters, right ventricle (RV) diameters, and RV wall thickness were significantly different between the groups (P<0.05). Pulmonary artery pressure (P<0.05) and pulmonary vascular resistance (P<0.05) were also seen to be higher in group 1 than those in group 2 and control group. CONCLUSIONS: Right ventricular diastolic dysfunction rate is high in chronic liver diseases. In the presence of HPS, right ventricular diastolic dysfunction is more remarkable in patients than those without HPS. Right ventricular diastolic dysfunction may result in dilatation and hypertrophy in the right heart.

Adult↗

Bridge to transplantation with the DeBakey VAD axial pump: a single center report.

AIMS: To report our experience with a left ventricular assist device axial pump as a bridge to transplantation: the DeBakey Ventricular Assist Device (VAD). METHODS: From February 1999 to February 2002, nine patients (among which eight males), with a mean age of 47 years, all in NYHA functional class IV, were proposed for a bridge to transplantation with the DeBakey VAD. Five patients had primary dilated cardiomyopathy, four had ischemic cardiomyopathy. All the patients had inotropic support prior to the intervention (dobutamine with a mean dose of 12 mcg/kg per min), six had an intra-aortic counterpulsation, four presented ventricular rhythm disorders. Interventions were performed through sternotomy alone (no need for an abdominal pocket) under extra-corporeal circulation on beating heart (except in one patient suffering from an apical thrombosis for which cardioplegic arrest was performed) as followed: implantation of the apical inflow cannula, tunneling of the percutaneous cable, implantation of the outflow graft under aortic side clamping, starting of the DeBakey VAD during CPB weaning-off. RESULTS: Mean support duration was 81+/-62 days (16-224 days). Eight reoperations were required (three for bleeding or cardiac tamponade, one for haemoperitoneum, one for aortic bifurcation thrombectomy, one for right ventricular assist device implantation, two for iterative replacements of the DeBakey VAD). A significant hemolysis was observed in two patients. No device infection or dysfunction were observed. Secondary recovery of a pulsed flow was observed either clinically or by Echo-Doppler in six patients. Five patients were transplanted, four died prior to transplantation (three from multi-organ failure on post-operative day 35, 16 and 50, respectively, and the last patient was found disconnected at day 109). CONCLUSIONS: The DeBakey VAD is at the origin of renewed interest for continuous flow assist devices. Still under evaluation, the advantages of miniaturization and facility of implantation of this new device seem to be promising.

Adult↗

A case of arrhythmogenic right ventricular dysplasia with left and right ventricular hypofunction in an elderly subject--long-term follow-up.

A 72-year-old man was admitted with palpitations caused by sustained ventricular tachycardia. He had been followed for 27 years, with admissions on 7 occasions, on account of ventricular tachycardias, atrial fibrillation, and congestive heart failure. Electrophysiologic examination detected 2 points of origin of ventricular tachycardia in the right and left ventricles. During the examination a myocardial infarction led to death. At autopsy, most areas of the right ventricle and the left ventricular apex were found to have been replaced by fatty tissue, pointing to severe fatty infiltration into the myocardium. Thus, this patient represented a unique cause of arrhythmogenic right ventricular dysplasia (ARVD) with severe fatty degeneration causing progression of ARVD. It is important that a diagnosis of ARVD is made carefully and rigorously in elderly subjects.

Aged↗

Primary cardiac malignant fibrous histiocytoma in the right ventricular infundibulum treated with a cavo-pulmonary shunt and coronary embolization.

A 51-year-old woman presented with progressive right ventricular infundibular wall thickening and outflow obstruction. She had had an aorto-coronary bypass for left main coronary artery disease 1 year after radiation therapy for left mammary cancer. Enhanced computed tomography showed a mass in the right ventricular free wall with no connection to the mediastinum; the tumor extended into the main pulmonary artery, but there was no other evidence of a primary or metastatic tumor. A biopsy specimen was obtained and based on the microscopic and immuno-histochemical findings (vimentin and Kp-1 positive) the diagnosis was primary cardiac malignant fibrous histiocytoma, which is very rare. A cavo-pulmonary artery connection lessened her symptoms, but embolization of the coronary artery to try and to reduce the mass had minimal effect. Four months after the tumor was diagnosed she died of extended pulmonary artery obstruction.

Arterial Occlusive Diseases↗

[Prevailing right ventricular myocardiopathy for previous myocarditis or arrhythmogenic dysplasia?].

A clinical case of a 10-year-old male patient is reported. His dilated and prevailing right ventricular myocardiopathy shows diagnostic difficulties between previous myocarditis etiology and arrhythmogenic dysplasia. As the elements are not pathognomonic of one or other cause, the increase of cardiac enzymes in subacute stage maybe tends to the supposition of previous myocarditis. Hence, the questioning that many cases labeled as arrhythmogenic dysplasia can truly correspond to the possibility of evolutional myocarditis. The controversial clinic management is disputable.

Arrhythmogenic Right Ventricular Dysplasia↗

[Right ventricular dilatation and intraventricular septal motion after surgical closure of atrial septal defect].

The persistence of right ventricular dilatation and paradoxical interventricular septal motion are two echocardiographic abnormalities rarely reported after surgical closure of atrial septal defects. The aim of this study was to identify the predictive factors of these abnormalities in the long-term and to study their functional consequences. One hundred and two patients aged 18 +/- 14 years (range 1-62 years) underwent closure of atrial septal defects. Thirty-five patients were under 10 years of age, 33 were 10 to 20 years of age and 34 were over 20. Fifty-six patients were female. The rhythm was sinus in the great majority of cases (97%). Three patients, all over 40 years of age, were in atrial fibrillation. Before surgery, right ventricular dilatation was observed in 95 patients (91.2%), paradoxical septal wall motion in 93 patients (91.2%), the ratio of pulmonary/systemic output was 2.7 +/- 0.6 (range 1.7 to 7.4) and over 2 in 90% of patients: pulmonary systolic pressure was 32.3 +/- 12 mmHg and over 40 mmHg in 18 patients (17.6%). Ninety-four patients were followed up regularly with a mean follow-up time of 5.5 +/- 3.6 years (1-14 years). The right ventricle remained dilated in 37 patients (39.4%) after surgery: the right ventricular dimension decreased from 36 +/- 1 to 27.8 +/- 6.2 mm (p = 0.001). The ratio of end diastolic right ventricular/left ventricular dimension also decreased from 1.07 +/- 0.31 to 0.56 +/- 0.12 (p = 0.0001). Multivariate analysis identified two predictive factors of persistent right ventricular dilatation: age > 40 years (p = 0.009) and a pulmonary/systemic flow ratio > 3 (p = 0.03). Interventricular septal wall motion remained paradoxical in 21 patients (22%). Multivariate analysis identified two predictive factors of persistent paradoxical septal motion: age > 40 years (p = 0.02) and systolic pulmonary pressures > 40 mmHg (p = 0.03). These abnormalities remained asymptomatic in all but two patients with persistent long-term hypertension and a residual atrial septal defect. The persistence of right ventricular dilatation and paradoxical septal motion was quite common, with older age at surgery, systolic pulmonary artery pressure > 40 mmHg and a ratio of pulmonary/systemic blood flow > 3, being predisposing factors. These abnormalities were clinically asymptomatic when isolated.

Adolescent↗

The right ventricle in mitral regurgitation: evaluation by electron beam tomography.

Mitral valve regurgitation affects heart ventricle volumes, which can be accurately measured by electron beam tomography (EBT). Most studies have focused on the left ventricle, but findings have failed to resolve some crucial clinical issues. We reviewed EBT studies of 35 patients with moderate mitral valve regurgitation but no ischemia, aortic valve regurgitation, or left ventricular failure to investigate the relationship between right ventricle volumes and ejection fraction and extent of mitral regurgitation. EBT in cine mode was performed during exercise and at rest in 839 patients from 1990-1994. Sixty-one of these showed evidence of significant mitral regurgitation, 35 of whom met the criteria for inclusion in this study. Left and right end-diastolic and end-systolic volumes were computed from the scans, and ventricular stroke volumes, ejection fractions, regurgitant stroke volume, and regurgitant fraction were computed from these values. Correlation coefficients between ventricular values and regurgitant fraction were computed. Regurgitant fraction was significantly and negatively associated with the ejection fraction in the right ventricle (r = -0.55, p < 0.001) but not in the left. Ten of the 35 patients studied had right ventricular ejection fractions of less than 45%. In addition, there was evidence for a subgroup of cases with moderate amounts of regurgitation and dilation of the right ventricle. It is concluded that reduced right ventricular ejection fraction is a common finding in patients with mitral regurgitation without left ventricular failure. It is most commonly observed in cases with large regurgitant fraction and/or with a large right ventricular end-diastolic volume/left ventricular end-diastolic volume ratio. Identification of mitral regurgitation patients with reduced right ventricular ejection fraction may have clinical significance, indicating a sub-group of patients with more severe disease prior to the development of left ventricular dysfunction.

Aged↗

Total right ventricular exclusion procedure: an operation for isolated congestive right ventricular failure.

OBJECTIVE: To prevent possible deleterious effects of right ventricular volume overload on cardiorespiratory function, we developed a total right ventricular exclusion procedure for the treatment of end-stage isolated congestive right ventricular failure. METHODS: Since 1996, this procedure has been performed in 5 patients in New York Heart Association functional class IV: 2 adults with arrhythmogenic right ventricular dysplasia and 3 children with Ebstein anomaly. The entire right ventricular free wall was resected along the atrioventricular groove and then parallel to the interventricular septum, sparing the pulmonary valve and a skeletonized right coronary artery. The orifice of the tricuspid valve was closed with either a polytetrafluoroethylene patch or with its leaflets. The defect of the right ventricular free wall was covered with a polytetrafluoroethylene patch in the 2 patients with arrhythmogenic right ventricular dysplasia and directly closed with the remnant of the free wall in the 3 children with Ebstein anomaly. After resection of a redundant right atrial wall, coronary sinus blood flow was rerouted into the left atrium through an atrial septal defect. A total cavopulmonary connection was constructed in 4 patients and a bidirectional superior cavopulmonary anastomosis in 1 infant. The heart was controlled with a DDD pacemaker in 3 patients. RESULTS: The patients were extubated at a mean of 14 hours postoperatively (range, 1-38 hours). There were no early or late deaths. At follow-up, ranging from 8 to 57 months, the mean cardiothoracic ratio had decreased from 74% +/- 7% before the operation to 52% +/- 6% (P <.01). All patients are in functional class I. Neither of the patients with arrhythmogenic right ventricular dysplasia have had attacks of ventricular tachycardia nor are they using antiarrhythmic medication. CONCLUSIONS: The total right ventricular exclusion procedure provides effective decompression of the lung, as well as the left ventricle, and may result in more effective volume loading of a surgically created single ventricle with increased systemic output. We believe that this new surgical option offers rescue treatment for isolated end-stage right ventricular failure in critically ill patients.

Adult↗

Right ventricular function in asymptomatic individuals with a systemic right ventricle.

BACKGROUND: In congenital heart defects where the morphologic right ventricle (RV) supports the systemic circulation, RV failure is common yet develops gradually. We hypothesized that patients who are asymptomatic may have unrecognized RV dysfunction. METHODS: Conventional and Doppler tissue/strain echocardiography were performed on consecutive patients with asymptomatic systemic RV caused by congenitally corrected transposition of the great arteries (ccTGA) and on age-matched control subjects. RV index of myocardial performance was measured using conventional echocardiography. Longitudinal tissue velocities, strain rate, and strain of the basal RV free wall were measured using Doppler tissue/strain echocardiography and compared with nonsystemic RV of the control subjects. RESULTS: Mean age was 39.5 +/- 14.6 (n = 13) and 36 +/- 24 (n = 10) years for ccTGA and control groups, respectively. Mean RV index of myocardial performance was higher in patients with ccTGA than in control subjects (0.66 +/- 0.25 vs 0.28 +/- 0.12, P < .001). Mean RV tissue displacement (10.8 +/- 4.5 vs 20.3 +/- 3.9 mm, P < .0001), peak systolic strain rate (-1.16 +/- 0.3 vs -2.23 +/- 0.9 s(-1), P = .005), and peak systolic strain (-17.3 +/- 8.0 vs -30.6 +/- 11.0%, P = .008) were significantly lower in patients with ccTGA compared with control subjects, respectively. CONCLUSION: Patients with asymptomatic ccTGA have quantifiable RV dysfunction by echocardiography.

Adult↗