Endomyocardial fibrosis: report of eight cases.

Endomyocardial fibrosis is a disease of unknown origin which has not previously been described in detail from the Middle East. The clinical, echocardiographic, hemodynamic and angiocardiographic findings in eight patients (five men and three women, mean age 38 years) are presented. Two patients had right-sided involvement, two had left-sided involvement and four had biventricular involvement. The presence of a small ventricle with obliteration of the apex and a large atrium is a two-dimensional echocardiographic finding highly suggestive of endomyocardial fibrosis. Hemodynamic characteristics of dip and plateau on ventricular pressure curves were present in six patients. Ventricular angiography was diagnostic in all cases. Endomyocardial biopsy yielded positive findings in three of six patients and is not essential for diagnosis.

One- to ten-year follow-up results of balloon angioplasty of native coarctation of the aorta in adolescents and adults.

OBJECTIVES: We attempted to evaluate the role of balloon angioplasty in the treatment of discrete coarctation of the aorta in adolescents and adults, with special emphasis on long-term results. BACKGROUND: Controversy persists over the use of balloon dilation for the treatment of native coarctation of the aorta. METHODS: Between July 1986 and January 1997, 43 consecutive adolescent and adult patients with discrete coarctation of the aorta underwent balloon angioplasty. One- to 10-year follow-up data of 37 patients, including results of cardiac catheterization and magnetic resonance imaging (MRI), form the basis of this study. RESULTS: No early or late deaths occurred. Balloon angioplasty produced a reduction in the peak to peak coarctation gradient from a mean +/- SD of 69 +/- 24 mm Hg (95% confidence interval [CI] 61 to 76) to 12 +/- 8 mm Hg (95% CI 10 to 14.8) (p < 0.001). Follow-up catheterization 12 months later (37 patients) revealed a residual gradient of 6.7 +/- 6 mm Hg (95% CI 4.6 to 8.9); 3 (7%) of 43 patients had suboptimal results with development of recoarctation, defined as peak gradient >20 mm Hg, with successful repeat angioplasty. A small aneurysm developed at the site of dilation in 3 (7%) of the 43 patients. MRI follow-up data 1 to 10.8 years (mean 5.2 +/- 2.7) after angioplasty (37 patients) revealed no new aneurysm or appreciable change in the size of the preexisting aneurysm in the three patients. The blood pressure had normalized without medication in 27 (73%) of 37 patients at follow-up examination. CONCLUSIONS: Balloon angioplasty is safe and effective and should be considered a viable alternative to operation for treatment of discrete coarctation of the aorta in adolescents and adults.

Hemodynamic evaluation of porcine bioprostheses in the mitral position by Doppler echocardiography.

Twenty-four patients with porcine bioprostheses in the mitral position were studied by Doppler echocardiography followed by cardiac catheterization within 24 hours. Doppler mean diastolic mitral valve gradient was calculated by a 3-point method and mitral valve area was determined by the pressure half-time method. Data from Doppler echocardiography and cardiac catheterization were compared. There was a strong correlation between Doppler echocardiography and catheterization-determined mean diastolic gradient: r = 0.9, standard error of estimate (SEE) = 1.4 mm/Hg (regression equation y = 0.63x + 1.41), p less than 0.001. There was also a strong correlation between Doppler echocardiography and catheterization-determined mitral valve area: r = 0.86, SEE = 0.18 cm2 (regression equation y = 0.64x + 0.52), p less than 0.001. Fourteen patients whose valvular function was considered normal by clinical evaluation had Doppler-calculated mean diastolic gradients of 4.5 to 9.5 mm Hg (mean 6.5 +/- 1.4); the Doppler-determined valve area was 1.15 to 2.0 cm2 (mean 1.54 +/- 0.3). Ten patients had a malfunctioning bioprosthesis, 7 had severe mitral regurgitation and 3 had stenosis. Valvular malfunction in all 10 patients was detected by Doppler echocardiography and confirmed by catheterization and angiocardiography. Nine patients underwent reoperation. Doppler hemodynamic evaluation of porcine bioprostheses in the mitral position provided noninvasive information comparable to that obtained by cardiac catheterization.

Anticoagulant therapy in children with prosthetic valves.

The purpose of this study was to evaluate the effectiveness and complications of several types of anticoagulant therapy in children with prosthetic valves. During a 7-year period ending April 1985, 130 children aged 1 to 19 years underwent left-sided valve replacement. Operative mortality was 3%, 5%, and 9%, respectively, for aortic, mitral, and aortic and mitral valve replacement. Among the 123 survivors, 32 (26%) had had aortic, 71 (58%) had had mitral, and 20 (16%) had had aortic and mitral valve replacement. Follow-up ranged from 2 months to 8.2 years, a total of 544 patient-years. The survivors were divided into three groups based on anticoagulant treatment: warfarin sodium, aspirin plus dipyridamole, and no anticoagulants. Among the patients who had aortic valve replacement, thromboembolic complications developed in 2.5% (2.5/100 patient-years) of the aspirin plus dipyridamole group and 5% of the group given no anticoagulants. Only the warfarin group (4%) experienced bleeding complications. Among the patients having mitral valve replacement, thromboembolic complications developed in 4% of the warfarin group, 3% of the aspirin plus dipyridamole group, and 11% of the no anticoagulant group. In addition, 2% of patients in the warfarin group experienced severe bleeding. Two fatal cerebrovascular accidents occurred, both in the aspirin plus dipyridamole group. Patients who received a mitral heterograft were not prescribed any anticoagulant medications, and no thromboembolic complications developed. Among patients having double-valve replacement, complications developed in 5% of the warfarin group and 27% of the group given no anticoagulants.(ABSTRACT TRUNCATED AT 250 WORDS)

Severe supravalvular aortic stenosis: successful surgical correction by extended aortoplasty.

Surgical treatment of severe discrete supravalvular aortic stenosis by placement of a patch graft across the narrowed area of aorta at one point has been associated with a substantial incidence of residual stenosis. Successful relief of a severe stenosis was achieved by a modification of a previously reported technique in which the stenotic area is expanded at two points. Use of this technique should be considered in cases of severe discrete supravalvular aortic stenosis.

The role of surgical ligation of patent ductus arteriosus in the era of the Rashkind device.

BACKGROUND: The role of surgery in managing patent ductus arteriosus (PDA) was studied in the era of the Rashkind double-umbrella device. METHODS: All 354 patients with PDA referred to our center in a 5-year period were included in this report. Of the 354 patients, 236 underwent cardiac catheterization with the intent of transcatheter PDA closure, and 118 had surgical intervention. RESULTS: In 46 (19.5%) of the 236 patients having cardiac catheterization, the procedure either was abandoned or failed. Color Doppler echocardiography demonstrated total occlusion of the ductus after 24 hours in 97 patients (41%) in the cardiac catheterization group. An additional 20 patients had no residual leaks at follow-up. Twenty other patients underwent reocclusion because of a residual shunt. Thus, of the 236 patients, 137 (58%) had successful complete closure of the PDA. Surgical PDA ligation was performed in 118 patients as the initial procedure and in 26 of the 46 patients in whom transcatheter closure was abandoned. If the remaining 20 patients in whom transcatheter closure failed are added to the 144 patients who underwent PDA ligation, the percentage having surgical intervention versus transcatheter occlusion is higher than 46%. CONCLUSIONS: Our data suggest that surgery plays a major role in the management of patients with PDA despite the advent of new interventional catheterization techniques.

The long-term effect of successful mitral balloon valvotomy on left atrial size.

BACKGROUND AND AIM OF THE STUDY: The study aim was to determine the extent of regression of left atrial (LA) enlargement following mitral balloon valvotomy (MBV) for mitral stenosis. METHODS: Data obtained from 205 patients before, and at a mean of 31.0 +/- 21.1 months (range: 6 to 86.3 months) after successful MBV were analyzed retrospectively. RESULTS: The invasively determined mitral valve area increased from 0.81 +/- 0.27 cm2 at baseline to 1.73 +/- 0.54 cm2 immediately after valvotomy (p <0.0001), and the mean mitral gradient fell from 15.6 +/- 5.3 to 5.4 +/- 2.5 mmHg (p <0.0001). Similar changes were noted in Doppler-determined mitral valve area (0.89 +/- 0.16 to 1.97 +/- 0.29 cm2; p <0.0001) and gradient (12.6 +/- 5.3 to 4.9 +/- 1.7 mmHg; p <0.0001). In comparison with baseline, significant (p <0.0001) reductions were noted at follow up in the echocardiographic anteroposterior (48.7 +/- 6.9 to 42.4 +/- 6.6 mm), superior-inferior (68.5 +/-8.1 to 59.6 +/- 8.2 mm) and medial-lateral LA dimension (51.2 +/- 6.7 to 44.1 +/- 7.7 mm) and calculated LA volume (91.6 +/- 29.1 to 60.7 +/- 23.8 cm3) Patients in atrial fibrillation had larger LA dimensions, but substantially smaller absolute and relative reduction in LA size at follow up than patients in sinus rhythm. Among patients with prevalvotomy LA enlargement, normalization of LA dimension at follow up was seen in 29.2% of patients in sinus rhythm, but in none of the 32 with atrial fibrillation. CONCLUSIONS: Successful MBV results in significant long-term reduction in LA size in most patients, but normalization of LA size is unusual.

Long-term results of pulmonary balloon valvulotomy in adult patients.

BACKGROUND AND AIM OF THE STUDY: The study aim was to define the long-term outcome of pulmonary balloon valvulotomy (PBV) in adult patients. METHODS: PBV was performed in 87 patients (46 females, 41 males; mean age 23+/-9 years; range: 15-54 years) with congenital pulmonary valve stenosis (PS). Intermediate follow up catheterization (mean 14.6+/-5.0; range: 6-24 months) was performed after PBV in 53 patients. Clinical and Doppler echocardiography examinations were carried out annually in 82 patients (mean 8.0+/-3.9; range: 2-15 years). RESULTS: There were no immediate or late deaths. The mean catheter peak pulmonary gradient (PG) before and immediately after PBV, and at intermediate follow up was 105+/-39, 34+/-26 (p <0.0001) and 17+/-14 (p <0.0001) mmHg, respectively. The corresponding values for right ventricular (RV) pressure were 125+/-38, 59+/-21 (p <0.0001) and 42+/-12 (p <0.0001) mmHg, respectively. The infundibular gradients immediately after PBV and at intermediate follow up were 31+/-23 and 14+/-9 mmHg (p <0.0001), whilst cardiac index improved from 2.68+/-0.73 to 3.1+/-0.4 l/min/m2 (p <0.05) at intermediate follow up. Doppler PG before PBV and at intermediate and long-term follow up were 91+/-33 (range 36-200) mmHg, 28+/-12 (range 10-60) mmHg (p <0.0001) and 26+/-11 mmHg (p = 0.2), respectively. New pulmonary regurgitation (PR) was noted in 21 patients (25%) after PBV. Five patients (6%) with a suboptimal result (immediate valve gradient > or =30 mmHg) developed restenosis and underwent repeat valvulotomy 6-12 months later using a larger balloon, and with satisfactory outcome. Moderate to severe tricuspid regurgitation (TR) in seven patients regressed after PBV. CONCLUSION: The long-term results of PBV in adults are excellent, with regression of concomitant, severe infundibular stenosis and/or severe TR. Hence, PBV should be considered as the treatment of choice for adult patients with PS.

Long-Term outcome of mitral balloon valvotomy in pregnant women.

BACKGROUND AND AIM OF THE STUDY: The study aim was to examine the long-term outcome (nine years) of mitral balloon valvotomy in pregnant patients with severe mitral stenosis. METHODS: Twenty-three patients with severe, symptomatic (NYHA class III/IV) mitral stenosis underwent mitral balloon valvotomy using an Inoue balloon technique during the second trimester of their pregnancy; mean follow up in 19 patients was 5.1 +/- 2.8 years (range: 1 to 9 years). RESULTS: The procedure was successful in all patients. Immediately after valvotomy, the Doppler-derived mitral valve area increased from 0.90 +/- 0.18 to 1.97 +/- 0.36 cm2 (p <0.0001), and the transmitral mean gradient decreased from 15.7 +/- 4.7 to 5.5 +/- 1.6 mmHg (p <0.0001). Four patients had mild worsening of mitral regurgitation, and six developed insignificant interatrial communication immediately after valvotomy. There was no other morbidity or mortality. Patients showed a significant improvement in mean NYHA class, from 3.0 +/- 0.1 to 1.0 +/- 0.02 (p <0.001). Twenty-two patients had normal deliveries; one cesarean section in week 36 resulted in stillbirth. No developmental abnormalities were seen in the babies. At long-term follow up of mothers, the mitral valve area was 1.8 +/- 0.52 cm2; restenosis developed in three patients (16%). One baby died at one week from sudden infant death syndrome, and one at eight months, from pneumonia. All other children showed normal growth, development and speech for their age. CONCLUSION: Mitral balloon valvotomy using the Inoue balloon technique can provide satisfactory immediate relief and long-term outcome in pregnant patients with severe mitral stenosis.

Total cavopulmonary connection for right ventricular endomyocardial fibrosis.

Endomyocardial fibrosis is an endemic problem in tropical countries and is characterised by ventricular cavity obliteration, decreased ventricular compliance, and atrioventricular valve regurgitation. We report on a patient with right ventricular endomyocardial fibrosis resulting in obliteration of the cavity and tricuspid regurgitation treated successfully by total cavopulmonary connection and exclusion of the right ventricle.

Double-chambered right ventricle in 73 patients: spectrum of the disease and surgical results of transatrial repair.

OBJECTIVE: To review the spectrum of double-chambered right ventricle (DCRV) and the outcome of surgical repair in patients diagnosed between February 1988 and March 1999. DESIGN: The charts of patients with DCRV were studied. SETTING: Tertiary care hospital. PATIENTS AND METHODS: A total of 73 patients were identified. Sixty-nine underwent surgical repair, while four are awaiting surgery. The repair was through a transatrial approach in 61 patients, while in eight an additional ventriculotomy was performed. MAIN RESULTS: An associated ventricular septal defect (VSD) was present in 56 of 73 patients (77%). These patients were significantly younger (P<0.05) than the 17 patients without a VSD. Among patients with a VSD, the 31 requiring patch closure were significantly younger than the 25 patients having direct closure. Five older patients among those with intact septum had impaired right ventricular (RV) function as well as higher intraventricular gradients. At surgery the intraventricular obstruction was relieved by myomectomy. There was no hospital or late mortality. Following surgery, at a mean follow-up of 13.6 months, no increase in the intraventricular gradient was detected by Doppler echocardiography. CONCLUSIONS: The development of DCRV is associated with VSD in early life. The probability of the presence of a VSD decreases with age. The disease is progressive, resulting in increased intracavitary gradient within the RV and in RV impairment if it is not treated in a timely fashion. Transatrial repair is safe with excellent midterm results. In the presence of high gradients within the RV, a ventriculotomy may be necessary to obtain acceptable results.

Mitral balloon valvotomy using the Inoue balloon technique for selected patients with severe pliable rheumatic mitral valve stenosis: immediate and short-term results.

We selected 40 patients with severe symptomatic rheumatic mitral stenosis for balloon valvotomy using the Inoue balloon technique. The patients' mean age was 31 +/- 14 years and there were 24 females and 16 males. The patients were selected according to the following echo/Doppler criteria; 1. Severe mitral stenosis, i. e. mitral valve area (MVA) less than 1.1 cm2; 2. pliable anterior mitral valve leaflet; 3. absence of calcification of the mitral commissures and 4. absence of significant subvalvular mitral valve disease (Block echo score less than 8). We failed to cross the mitral valve in three cases and repeat attempts in two patients with higher transeptal puncture was successful. Thirty-nine procedures were technically successful (98%). There were no complications. We used an Inoue balloon size 24-30 mm using echo/Doppler guided stepwise mitral dilatation. After mitral balloon valvotomy, the MVA increased from 0.8 +/- 0.2 to 1.7 +/- 0.5 cm2 (p less than 0.001). Five patients developed mild mitral regurgitation and in one patient the degree of mitral regurgitation increased from mild to moderate. The mean mitral valve area 48 hours after the procedure measured 1.9 +/- 0.4 cm2 (echo/Doppler); one patient (2.5%) had residual mitral stenosis (MVA less than 1.5 cm2). At six weeks follow-up study the mean mitral valve area was 1.9 +/- 0.5 cm2 (Echo/Doppler), with no restenosis. We conclude that in selected cases of severe pliable mitral stenosis, the Inoue balloon technique achieves a greater than 100% increase of the MVA, without inducing significant iatrogenic mitral regurgitation or residual stenosis.

Endomyocardial fibrosis: Clinical, echocardiographic and hemodynamic findings.

Endomyocardial fibrosis (EMF) is a disease of unknown origin. It was first described by Davies in Uganda in 1948. The clinical enchocardiographic, and hemodynamic findings in 18 patients are presented. Six patients had right-sided involvement, four had left-sided involvement and eight had biventricular involvement. The presence of a small ventricle with obliteration of the apex and a large atrium, diagnosed by two-dimensional echocardiography, is highly suggestive of endomyocardial fibrosis. Ventricular angiography was diagnostic in 17 out of 18 cases. Endomyocardial biopsy yielded positive findings in three out of seven patients and is not essential for diagnosis. Two patients suffered a cerebral embolism. Six patients underwent surgery with good results in three patients.

The role of selenium deficiency in dilated cardiomyopathy in Saudi Arabia.

BACKGROUND: Selenium deficiency is implicated in the etiology of endemic juvenile dilated cardiomyopathy in China, and in sporadic cases in other countries. The aim of this study was to evaluate the role of selenium deficiency in the pathophysiology of dilated cardiomyopathy in the Saudi Arabian population. PATIENTS AND METHODS: Plasma and urine selenium concentrations from 72 Saudi patients with confirmed dilated cardiomyopathy were compared with corresponding values from 70 control subjects of the same national origin who had normal ventricular function. RESULTS: Plasma and urine selenium concentrations (mean+/-SD) were 1.347plusmn;0.45 and 0.49+/-0.37 micromol/L, respectively, for the patient group, and 1.32+/-0.41 and 0.60+/-0.41 micromol/L, respectively, for the control group. The differences in the values between the two groups were statistically insignificant. CONCLUSION: In the Saudi population, dilated cardiomyopathy is not caused by selenium deficiency.

Long term clinical and echocardiographic results of mitral balloon valvotomy in children and adolescents.

AIMS: To assess the safety, efficacy, and long term results of mitral balloon valvotomy (MBV) for rheumatic mitral stenosis in children and adolescents in comparison to adults. METHODS: The results of 468 patients with mitral stenosis who underwent successful MBV and were followed up for 0.5-13 years were analysed. Patients were divided according to age at the time of MBV into group 1 consisting of 84 patients < or = 20 years of age (children and adolescents) and group 2 that included 384 patients, age > 20 (adults). RESULTS: Patients in group 1 had a lower mitral echo score (mean (SD) 7.5 (1.3) v 8 (1.1), p < 0.001), smaller Doppler mitral valve area (MVA) (0.84 (0.17) v 0.92 (0.18) cm2, p < 0.001), and higher Doppler mitral valve gradient (15.0 (5.3) v 12.7 (4.5) mm Hg, p < 0.001) than group 2. Immediately after MBV group 1 had larger MVA, whether measured by Doppler (2.0 (0.30) v 1.96 (0.28) cm2, p < 0.05) or by catheter (2.0 (0.59) v 1.8 (0.52) cm2, p < 0.001), and similar complication rates, compared to group 2. After a mean follow up of 5 (3.5) years there was no significant difference between groups 1 and 2 in the incidence of restenosis (14.3% v 16.1%, NS). Event-free survival rates at 5, 10, and 12.5 years were 93%, 79%, and 79% for group 1 and 94%, 90%, and 84% for group 2 (p = 0.18). CONCLUSIONS: MBV is safe and effective in children and adolescents with rheumatic mitral stenosis. It provides better immediate results than in adults and excellent long term results that are comparable to those seen in adults.

Hydatid disease of the heart.

A case of hydatid cyst of the heart is described, in which the diagnosis was made at coronary angiography; a new angiographic sign is described.

Long-term results of balloon pulmonary valvuloplasty of valvar pulmonic stenosis.

The purpose of this paper is to document long-term results of percutaneous balloon pulmonary valvuloplasty. Forty-one patients, aged 7 days to 20 years, underwent pulmonary valvuloplasty over a 3 1/2-year period ending in April, 1987. Balloon valvuloplasty resulted in immediate reduction in the pulmonary valvar pressure gradient from 92 +/- 45 to 30 +/- 22 mm Hg (p less than 0.001). Follow-up (3 to 34 months) clinical, ECG, and echo Doppler data were available in 35 patients. Follow-up (6 to 34 months) cardiac catheterization data were available in 29 of the 35 patients. Short ejection systolic murmurs were heard in all 35, but an early diastolic decrescendo murmur was heard in only 12 patients. Based on the catheterization and Doppler data, the patients were divided into two groups: group I (30 patients) with excellent results and group II (five patients) with poor results (gradients greater than 50 mm Hg). In group I ECG right ventricular hypertrophy regressed. The echocardiographic right ventricular end-diastolic dimension (21 +/- 6 vs 15.9 +/- 4.6 mm) decreased (p less than 0.001) while the left ventricular dimension increased (p less than 0.02). Peak Doppler flow velocity in the main pulmonary artery fell from 4.0 +/- 0.8 m/sec to 2.3 +/- 0.5 m/sec (p less than 0.001). Doppler evidence for pulmonary insufficiency was present in 21 patients. Catheterization-determined pulmonary valvar gradients (24 patients) also fell from 95.6 +/- 50.3 mm Hg to 18.3 +/- 12.5 mm Hg (p less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

[Transcatheter obliteration of patent ductus arteriosus in young adults with the Rashkind occluder]. / Transkatheter-Verschulss des offenen Ductus arteriosus bei jungen Erwachsenen mit dem Rashkind-Occluder.

Fourteen young adult patients between 14 and 29 years of age underwent 15 procedures of transcatheter occlusion of patent ductus arteriosus. In all patients but one, the attempt to implant the device was successful (92%). In one patient the ductus was judged to be too large for occlusion and the patient was sent for surgery. In no patient did the device embolize. A total of 6/13 (46%) patients was discharged home with no residual shunt. Follow-up data (between 3 and 18 months) are available in 10/13 patients. To date, 9/13 (70%) had total occlusion of their duct, in one patient after reocclusion.

Regression of infundibular pulmonary stenosis after successful balloon pulmonary valvuloplasty in adults.

Between July 1985 and March 1988, 22 adult patients with congenital pulmonary stenosis underwent balloon pulmonary valvuloplasty. There were 10 males and 12 females aged 16-45 (average 25 +/- 9.9) years. All patients had additional mild to severe infundibular stenosis; 16 were restudied 6-36 (mean 12.6) months later by repeat catheterization. Student's t-test was used for comparison of data. Right ventricular (RV) systolic pressure before dilatation was 84-196 (mean 129 +/- 32.3) mm Hg, and the peak pulmonary gradient (PPG) was 60-176 (mean 111 +/- 33.2) mm Hg immediately after dilatation. The RV systolic pressure dropped to 32-140 (mean 59.2 +/- 27) (P less than 0.001); and PPG dropped to 10-113 (mean 37.8 +/- 26.4) (P less than 0.001), and the infundibular gradient ranged from 8 to 113 (mean 35.1 +/- 25.8) mm Hg. The infundibular diameter, before dilatation, ranged from 2 to 15 (mean 9.5 +/- 4) mm Hg. At repeat catheterization, the RV systolic pressure dropped further to 33-66 (mean 42.8 +/- 9.7) mm Hg and the PPG was reduced to 0-48 (mean 18.4 +/- 10.9) mm Hg (P less than 0.001). The infundibular gradient regressed to 0-34 (mean 15 +/- 8.8) mm Hg (P less than 0.001). The infundibular diameter increased to 8-25 (mean 15.8 +/- 5.4) (P less than 0.001). It is concluded that moderate to severe infundibular stenosis, in adults, can regress after successful pulmonary valvuloplasty.