Clinical and echocardiographic assessment of a right-to-left shunt across an atrial septal defect secondary to tricuspid regurgitation.

OBJECTIVE: Cyanosis in conjunction with atrial septal defect does not necessarily indicate the Eisenmenger syndrome. Exceptionally, the right-to-left shunt may result from tricuspid regurgitant flow and its unusual direction. METHODS: We describe thirteen patients with atrial septal defect and accompanying tricuspid regurgitation. In all of them, the tricuspid regurgitant jet was oriented towards the interatrial septum. This was found on the basis of echocardiographic examination and subsequent frame-by-frame analysis of videotape. RESULTS: Of thirteen patients, cyanosis was observed in 7 (53%). Among those seven, three had moderate pulmonary hypertension (pulmonary artery systolic pressure (PASP) between 40 and 60 mm Hg). The remaining four patients with marked pulmonary hypertension (PASP > 60 mm Hg) underwent cardiac catheterization, which demonstrated low values of pulmonary artery resistance. Except for the one patient with a history of the cerebral embolic event, all were qualified for cardiac surgery. In the postoperative observation they were uneventful and showed clinical recovery and echocardiographic improvement. CONCLUSIONS: Appreciable arterial desaturation and cyanosis in patients with ASD, regarded as uncomplicated, should be followed by careful investigation for the direction of tricuspid regurgitant flow.

Transvenous closure of moderate and large secundum atrial septal defects in adults using the Amplatzer septal occluder.

The aim was to determine the feasibility of using the Amplatzer septal occluder for closure of moderate and large secundum atrial septal defects in adults. Fifty patients aged 16-76 years (mean +/- SD, 40 +/- 15.5), underwent successful device implantation. Flow ratios of 1.4-8.5 (mean +/- SD, 2.6 +/- 1.6) were calculated. The defects were: centrally placed (n = 31), antero-superior with partial or total deficiency of aortic rim (n = 19), multiple (n = 3) and with aneurysmal septum (n = 23). They measured 4-25 mm (median 14) on echocardiography and balloon sized 7-31 mm (median 19.5). Devices of 7-34 mm (median 20) were implanted. Patient follow up for 1 month (50/50 patients), 3 months (40/50) and 12 months (13/50), achieved respective rates of 90%, 92% and 98% of complete occlusion. In one patient a transient atrioventricular block (2:1) developed, and one had a transient STT elevation. One female had an episode of 30 min loss of vision over the lateral aspect of the left eye 3 months after implantation. In conclusion, transvenous occlusion of secundum atrial septal defects with the Amplatzer septal occluder in adults is safe, and can be performed without significant complications. Large defects, defects with a very deficient or absent aortic rim, defects with an aneurysmal septum as well as some multiple defects can be closed with an almost 100% early complete occlusion rate. This makes the procedure an alternative to surgery for selected adult patients.

[Analysis of risk factors of the paroxysmal atrial fibrillation after valve replacement in patients with aortic stenosis]. / Napadowe migotanie przedsionków po leczeniu operacyjnym zwezenia zastawki aortalnej--ocena czynników ryzyka.

UNLABELLED: Paroxysmal atrial fibrillation (PAF) is a frequent complication (10-60% of pts) after cardiac surgery. In our study we analyze the influence of clinical, echocardiographic and 24 h ecg parameters on the risk of postoperative PAF in 266 pts with aortic stenosis (88 women and 178 men; mean age 58 +/- 10). PAF was observed in 74 (28%) patients. Statistically significant factors of risk of PAF were (univariate analysis): age-relative risk 1.08 (1.04-1.11), history of PAF--4.3 (1.4-12.5), more than 100 supraventricular ectopic beats during 24 h ecg--2.9 (1.6-5.1), presence of SVT during 24 h ecg--2.6 (1.5-4.5) and presence of SVT > 140/min--relative risk 3.5 (1.8-6.7). Left atrium diameter and coronary artery bypass grafting during valve replacement had no impact on the risk of PAF. In multivariate analysis three factors remained significant--age, history of PAF and presence of SVT > 140/min during 24 h ecg. In discriminant analysis this model of 3 factors enabled the correct risk assessment in 72% of patients. CONCLUSIONS: 1. The factors that increase the risk of postoperative PAF in pts with aortic stenosis are: age, history of PAF and presence of SVT > 140/min during preoperative 24 h ecg. 2. Postoperative PAF is not related to left atrium diameter in this group of patients. 3. Coronary artery bypass grafting during aortic valve replacement does not increase the risk of PAF.

Myocardial perfusion and function of the systemic right ventricle in patients after atrial switch procedure for complete transposition: long-term follow-up.

OBJECTIVES: Our purpose was to assess the right ventricular (RV) function and identify patients with RV impairment long after the Mustard or Senning operation. BACKGROUND: Systemic ventricular failure can cause myocardial perfusion abnormalities in thallium scintigraphy correlating with hemodynamic deterioration. METHODS: Myocardial perfusion at rest and at peak exercise was assessed in 61 patients, aged 7 to 23 years in mean time 10.0 +/- 2.9 years after surgery using technetium-99m methoxyisobutyl isonitrile single-photon emission computed tomography. Ventricular function was assessed by first-pass radionuclide angiography at rest. Exercise capacity was determined with a modified Bruce protocol. RESULTS: The mean RV ejection fraction was 36.1 +/- 7.7%, and left ventricular (LV) ejection fraction was 52.1 +/- 9.4%. Moderate or severe perfusion abnormalities on the rest scan were observed in 20 patients (33%). On exercise perfusion worsened in another 13 patients (21.3%). Patients with perfusion defects on stress scan had significantly lower RV and LV ejection fraction (33.2 vs. 39.4%; p = 0.002 and 49.2 vs. 55.5%; p = 0.01, respectively). They were also older (16.6 vs. 13.0 years; p = 0.002), operated on at an older age (4.0 vs. 2.4 years; p = 0.05) and had longer follow-up (12.5 vs. 10.5 years; p = 0.003). CONCLUSIONS: Myocardial perfusion defects are common findings in patients in long-term follow-up after atrial switch operation. Despite excellent exercise tolerance, the extent of myocardial perfusion abnormalities correlated well with impaired RV and LV function, and greater perfusion defects were seen more frequently in older patients with longer follow-up. It is likely that myocardial perfusion defects could be a sensitive predictor of systemic ventricular impairment.

Left ventricular outflow tract gradient decrease with non-surgical myocardial reduction improves exercise capacity in patients with hypertrophic obstructive cardiomyopathy.

OBJECTIVES: This study was undertaken to evaluate mid-term clinical results of non-surgical myocardial reduction in patients with hypertrophic obstructive cardiomyopathy. METHODS: Twenty-five patients with left ventricular outflow tract obstruction (mean gradient of 84. 54+/-31.38 mmHg) and symptoms of dyspnoea, angina and/or syncope were treated with non-surgical myocardial reduction. The patients were followed-up for a mean period of 10.44+/-1.8 months. In all patients clinical examination with echocardiography was repeated after every 3 months of follow-up, and a symptom-limited treadmill test was repeated at the 6 month follow-up. Eighteen patients underwent simultaneous respiratory gas analysis. RESULTS: Clinical follow-up examinations were achieved in all 25 patients. Persistent left ventricular outflow tract gradient reduction was seen in 23 patients. Seventeen patients had a reduction of left ventricular outflow tract gradient >50% of baseline value. Twenty patients showed a clinical improvement from 2.8+/-0.5 up to 1.2+/-0.5 NYHA class (P<0.001). The clinical improvement was matched by an improvement in objective measures of exercise capacity in patients with significant left ventricular outflow tract gradient reduction. Exercise time increased from 571.9+/-192.2 to 703.5+/-175.4 s, P<0. 001, and peak VO(2)increased from 14.6+/-5.2 to 20.5+/-8.6 ml. kg(-1)min(-1), P<0.05. CONCLUSION: Significant left ventricular outflow tract gradient reduction with exercise capacity improvement was achieved in the majority of patients treated with non-surgical myocardial reduction. We recommend this method as an alternative to surgery for symptomatic patients with hypertrophic obstructive cardiomyopathy.

[Transcatheter closure of patent foramen ovale in patients after cryptogenic stroke]. / Przeznaczyniowe zamykanie droznego otworu owalnego u chorych po udarze niedokrwiennym.

Paradoxical embolism through a patent foramen (PFO) is a possible mechanism of ischaemic stroke in patients with cryptogenic stroke. Occlusion of PFO in such patients is considered by some authors as most effective in stroke prevention. We present our initial experience with transcatheter closure of PFO with the new self-expanding device--the Amplatzer PFO occluder in three young patients (age < 50 years). Each of them experienced at least one ischaemic stroke episode, without a left heart or carotid source and each had an interatrial communication with right-to-left shunting during Valsalva manoeuvre on echocardiography. The PFO's were closed completely without complications, under transoesophageal echo guidance in general anaesthesia. Complete closure was confirmed at one-month follow-up echocardiogram in each patient. No repeat cerebral accidents occurred at that time. The procedures were relatively easy and the clear presentation of the implant on TEE and fluoroscopy, made implantation fully controlled. The unique feature of the device is, that until release it can easily be retrieved, repositioned or removed. Transcatheter closure of PFO with the Amplatzer PFO occluder may become the new therapeutic option for patients with cryptogenic stroke and presumed paradoxical embolism.

Mechanical valve replacement in congenital heart disease in children.

BACKGROUND AND AIM OF THE STUDY: The study aim was to analyze predictive factors of long-term results after mechanical heart valve replacement in children. METHODS: Forty-four patients (19 males, 25 females; mean age 8.9+/-3.9 years, median 7.0 years, range: 1.3 to 15 years) underwent heart valve replacement with mechanical prostheses. Of these patients, 25 had left atrioventricular valve replacement (LavVR) (18 mitral, six tricuspid in corrected transposition of the great arteries (TGA), one common in a univentricular heart), 13 had aortic valve replacement (AVR) and six had tricuspid valve replacement (TVR). The etiology of the valvular disease was congenital in all patients, and complicated by infective endocarditis in seven (16%). Fifteen patients had undergone previous procedures and 16 required simultaneous repair of associated lesions. The mean size of the implanted prosthesis was 26 mm (range: 19-29 mm) for LavVR, 29.7 mm (range: 23-33 mm) for TVR, and 21.9 mm (range: 19-25 mm) for AVR. Postoperatively, all patients received oral anticoagulation. The mean follow up was 6.8+/-3.5 years (total 290 patient-years). RESULTS: There was no early mortality, but three patients (7%) died later; all late deaths occurred in patients with LavVR. There were two sudden deaths, both in patients with complex congenital heart disease and heart failure (before the ACE inhibitor era), and one valve-related death from thrombotic occlusion of a mitral prosthesis. Five patients were reoperated on; three for patient-prosthesis mismatch, one for periprosthetic leak, and one for aortic dissection due to Marfan's syndrome. Thrombotic obstruction occurred in three patients; two in the tricuspid position were treated successfully with thrombolysis, but one in the left atrioventricular position proved fatal. After seven years, the survival rate was 93.4%. CONCLUSIONS: Mechanical heart valve replacement in children aged over one year with congenital heart disease can be performed with satisfactory early and late results. Mechanical valves of >23 mm diameter in the atrioventricular position in the systemic ventricle, and >21 mm in the aortic orifice, can offer excellent long-lasting hemodynamic performance. However, mechanical valves in the tricuspid position are prone to develop thrombotic occlusion.

[Usefulness of dobutamine stress echocardiography in qualification of patients to angioplasty of infarct related artery]. / Przydatnosc echokardiograficznego testu dobutaminowego w kwalifikacji chorych do zabiegu udroznienia tetnicy odpowiedzialnej za zawal.

UNLABELLED: Late angioplasty of an infarct-related artery (IRA) performed weeks or months after myocardial infarction (MI) may restore blood flow in IRA and would result in improvement of regional wall motion. This study was designed to assess dobutamine stress echocardiography (DSE) in predicting the improvement of regional left ventricular (LV) function in patients after late PTCA of IRA. MATERIAL AND METHODS: 44 patients (36 M 8 F, mean age 54 +/- 7 year) who had a MI 15 +/- +/- 8 weeks earlier, were qualified to angioplasty of IRA when DSE showed the change in wall motion at the infarct zone--improved during low dose infusion (5-10 micrograms/kg/min) and/or worsened during a high dose dobutamine (up to 40 micrograms/kg/min). Regional wall motion was assessed by DSE performed at 2-7 days and 6-month after successful angioplasty. Wall motion score index (WMSI) decreased from 1.51 +/- 0.29 at rest to 1.31 +/- 0.28 at low-dose dobutamine infusion (p > 0.001) before angioplasty. Early after PTCA regional wall motion improves and baseline, exercise and rest values of WMSI decreased, compared with values before angioplasty. At baseline echocardiography 248 of segments were dyssynergic (112 hypokinetic, 125 akinetic and 10 dyskinetic). Viability during DSE was more frequent in segments with biphasic reaction during DSE than in segments showed monophasic reaction. In 34 patients control angiography (CA) was performed 6 month after successful PTCA. In this selected group CA showed significant restenosis of IRA in 12 pts (35% =, 9 of them showed positive DSE. Their base WMSI increased from 1.36 +/- 0.28 to 1.47 +/- 0.30 and peak stress WMSI from 1.32 +/- 0.29 to 1.60 +/- 0.30 (p < 0.001), respectively, between the exam just after PTCA comparing with the exam which detected restenosis. CONCLUSIONS: In some patients with infarction and a narrowed or occluded IRA the myocardium remains viable for a prolonged period. Improvement of wall motion during low-dose DSE and/or worsened during high dose DSE at infarct zone is a very useful tool in predicting of viable myocardium at infarct zone. DSE is an excellent method to demonstrate an immediate improvement in regional LV dysfunction after angioplasty of IRA. At 6 months' follow-up restenosis of IRA is often seen.

[Long-term follow-up of supraventricular tachyarrhythmia in adult patients with secundum atrial septal defect before and after surgery]. / Tachyarytmie nadkomorowe u doroslych pacjentów z ubytkiem przegrody miedzyprzedsionkowej typu II przed operacja i w obserwacjach odleglych.

The frequency of arrhythmias was determined pre- and postoperatively in adult patients with secundum atrial septal defect. The study group consisted of 224 pts who had undergone surgical repair of ASD II in between 1987-1993. The mean postoperative follow-up was 7.5 y. The group (160 F, 64 M, aged 17-66 y) was divided on 3 subgroups: I--age under 20 y--27 pts, II--20-40 y--131 pts, III--over 40 y--66 pts. We analysed pre- and postoperative parameters: 1) arrhythmias in the 24-h Holter recording, 2) pulmonary hypertension (PASP-Doppler), 3) coexisting diseases, 4) functional class of heart failure (NYHA). Most common abnormalities found were supraventricular tachyarrhythmias (22.8 pre- and 40.6% postoperatively). There was significant increase in frequency of arrhythmias after surgery in the subgroups II and III. Arrhythmias were most frequent in patients with coexisting arrhythmogenic diseases.

[Pulmonary hypertension in adult patients with secundum atrial septal defect before surgery and in long-term follow-up]. / Nadcisnienie plucne u doroslych pacjentów z ubytkiem przegrody miedzyprzedsionkowej typu II przed operacja i w obserwacjach odleglych.

The aim of the study was to determine pre- and postoperative systolic pulmonary pressure in adult patients with ASD II. The study group consisted of 224 pts who had undergone surgical repair of ASD II in between 1987-1993. The mean postoperative follow-up was 7.5 y. The group (160 F, 66 M aged 17-66 years) was divided on 3 subgroups: age under 20 y--27 pts; 20-40 y--131 pts; and older then 40 y--66 pts. We analysed pre- and postoperative parameters: pulmonary hypertension (PASP-Doppler) and functional class (NYHA). Preoperative pulmonary pressure was 35-50 mm Hg in 69 pts (30.8%) of the group, and over 50 mm Hg in 29 (12.9%). In long-term follow-up of 98 (43.8%) operated patients with coexisting pulmonary hypertension, significant reduction of pulmonary pressure was observed in all patients, even in patients over 40 y. Functional class of heart failure NYXHA improved as well in all patients.

Long-term follow-up results of balloon valvuloplasty for congenital aortic stenosis: predictors of late outcome.

AIMS: Long-term follow-up of patients with aortic valve stenosis undergoing balloon valvuloplasty was evaluated with respect to survival, the need for repeat intervention and factors predicting late outcome. METHODS AND RESULTS: Forty-five patients between 3.5 to 23 years old (mean 11.7 +/- 4.5) were followed for 62 +/- 30 months (range 11-122). The transvalvar aortic gradient decreased from 84 +/- 20 to 36 +/- 10 mmHg (p < 0.001) and remained significantly lower (50 +/- 26 mmHg; p < 0.001) at follow-up. At that time, 10 patients (including 4 with significant valve incompetence) had gradients >/= 60 mmHg. The procedure resulted in significant valve incompetence (grade >/= 3) in 8 patients (17.8%). There was a progression of incompetence and 13 patients (28.9%) had significant regurgitation at follow-up. All survived. Fifteen patients (33.3%) required re-intervention 51 +/- 24 months after valvuloplasty. The indications were: aortic stenosis in 5 patients; regurgitation in 6 patients; and stenosis with regurgitation in 4 patients. Actuarial freedom from re-intervention at 2, 4, 6 and 8 years was 96%, 88%, 61% and 56% of patients, respectively. The residual post-valvuloplasty gradient was the only predictor of re-intervention for valve stenosis (odds ratio = 3.2 for every 10 mmHg gradient increase; p = 0.017). A residual post-valvuloplasty gradient >/= 40 mmHg increased the relative risk of re-intervention sixfold. The immediate post-valvuloplasty aortic regurgitation grade was the only risk factor of re-intervention for regurgitation (odds ratio = 34 for every incompetence degree increase; p = 0.0019). Incompetence grade >/= 2 increased the risk of re-intervention tenfold. CONCLUSIONS: Valvuloplasty carries the risk of development of valve incompetence, which progresses with time. Some patients develop restenosis. The

Captopril or nifedipine? Comparison of rest and exercise acute effects and long-term therapy in chronic isolated asymptomatic moderate to severe aortic regurgitation.

UNLABELLED: The effects of both single and long-term oral captopril or nifedipine treatment on cardiac parameters at rest and during exercise in patients with moderate to severe aortic regurgitation was investigated. METHODS: Thirty-one asymptomatic patients with chronic, isolated, previously untreated, moderate to severe aortic regurgitation (AR) of mean grade 3.1 +/- 0.6, had left ventricular end-diastolic diameter (LVEDD) 64 +/- 5 mm, left ventricular end-systolic diameter (LVESD) 41 +/- 5 mm, ejection fraction (EF) 66 +/- 6% and fractional shortening (FS) 37 +/- 5% measured by echo-Doppler. Bedside Swan-Ganz measurements at rest and at peak exercise (75 W) were conducted before (baseline) and at 75-90 min after oral administration of 20 mg nifedipine. Repeat testing was performed 24 h later, at 75-90 min after oral administration of 25 mg captopril. RESULTS: At rest, nifedipine significantly reduced systemic vascular resistance (SVR) compared with baseline (704 +/- 152 versus 880 +/- 216 dynes.s.cm-5; p < 0.0001) and captopril treatment (800 +/- 176 dynes.s.cm-5; p < 0.0001). Despite significant improvement of effective left ventricular (LV) stroke volume (LVSVef) after both nifedipine and captopril over baseline (103 +/- 20 ml), LVSVef did not differ between nifedipine and captopril (110 +/- 17 versus 110 +/- 22 ml; NS). Nifedipine significantly increased effective cardiac output (COef) from baseline (6.7 +/- 1.3 l/min) to 8.2 +/- 1.5 l/min; p < 0.0001, but this was due to an increase in heart rate (HR) (66 +/- 10 versus 75 +/- 1 beats/min; p < 0.0001). In contrast, captopril affected neither COef nor HR. In addition, captopril reduced pulmonary capillary wedge pressure (PCWP) more than nifedipine (8.7 +/- 2.5 versus 11 +/- 2.9 mmHg; p < 0.0001). At exercise, both drugs caused similar reductions in blood pressure and systemic vascular resistance (SVR). By comparison with exercise baseline, LVSVef was increased by captopril (139 +/- 24 versus 147 +/- 27 ml; p < 0.01) but was unchanged after nifedipine. Compared with baseline, nifedipine increased COef (from 14.4 +/- 2.0 to 15.5 +/- 2.1 l/min; p < 0.0001) due to a significantly higher HR, while COef and HR were unchanged after captopril. A smaller increase in PCWP was also seen after captopril than nifedipine and baseline (both p < 0.0001). After long-term therapy (33 +/- 12; range: 12 to 53 months) with captopril (75 mg/day, n = 13) or nifedipine (40 mg/day; n = 12) there was no change in LVESD, and in left ventricular EF and FS in either groups. None of the patients became symptomatic. Compared with baseline, captopril significantly reduced AR grade by 0.9 +/- 0.6 (p < 0.01), but not significantly so versus nifedipine. LVEDD was reduced in captopril patients by 4.0 +/- 2.6 mm (p < 0.0002), but not significantly so in nifedipine patients. LVEDD was normalized in five captopril patients, and in four treated with nifedipine. CONCLUSIONS: Single captopril treatment caused a greater hemodynamic improvement than nifedipine, notably during exercise; these findings were confirmed by long-term therapy with both drugs. Therefore, captopril may delay the development of left ventricular dysfunction and thus the time for corrective surgery.

Ischemic neurologic event in a child as a result of ventricular septal aneurysm.

Aneurysm of the interventricular septum without a shunt was found by means of transthoracic echocardiography in a 6-year-old boy with acute hemiparesis. Until the time of the incident, he had been healthy. The aneurysmal sac contained 2 clots, presumably resulting in the ischemic cerebral infarct. On the basis of the echocardiographic scan, successful surgery was performed. To the best of our knowledge, this is the first report describing such dramatic sequelae of interventricular septal aneurysm.

[Dobutamine stress echocardiography in the monitoring of post-angioplasty myocardial contraction and in detection of restenosis]. / Echograficzny test dobutaminowy w monitorowaniu zmian kurczliwosci po angioplastyce wiencowej i w rozpoznawaniu restenozy.

UNLABELLED: The expanding role of percutaneous transluminal coronary angioplasty (PTCA) in the selected patients with coronary artery disease (CAD), requires the noninvasive method for the assessment of the influence of PTCA on left ventricular function and the detection of restenosis. Forty two patients (pts) with angina, undergoing PTCA, were evaluated with dobutamine stress echocardiography (DSE) 1 day before, 2-7 days after, 3-4 months and 1 year after successful angioplasty. Two-dimensional echocardiographic images were collected during incremental doses of intravenous dobutamine infusion (up to 40 micrograms/kg/min). In 31.7% of examinations atropine was added (0.5-1 mg). Echocardiograms were assessed at baseline and during low- and high-dose of dobutamine infusion. An echocardiographic test positive for restenosis was defined as showing a new wall motion abnormality induced by dobutamine 3-4 months or 1 year after PTCA, compared to DSE 2-7 days after PTCA. Whereas before angioplasty, the wall motion score index (WMSI) increased from 1.40 +/- 0.34 at rest to 1.56 +/- 0.34 at peak stress (p < 0.0001); after angioplasty WMSI decreased from 1.29 +/- 0.30 at rest to 1.24 +/- 0.28 at peak (p < 0.0001). In 20 patients control angiography (CA) was performed because of positive DSE (11/20) or for clinical reasons only (9/20). In this selected group CA showed significant restenosis in 10 patients with high DSE sensitivity and specificity, 83% and 91% respectively. In this group peak stress WMSI increased from 1.21 just after PTCA to 1.60 during the examination detecting restenosis (p < 0.01). CONCLUSION: 1. A reduction in myocardial ischaemia, as assessed by DSE, is seen early after angioplasty. 2. Serial DSE records changes in regional function, thus being a valuable tool in PTCA follow up.

Increased frequency of organ-specific cardiac antibodies in healthy relatives of patients with dilated cardiomyopathy: evidence for autoimmunity in Polish families.

BACKGROUND AND HYPOTHESIS: Autoantibodies represent markers of autoimmune involvement and are found with increased frequency in patients and their symptom-free relatives at risk compared with normal controls. Cardiac-specific autoantibodies, detected by immunofluorescence, were found in 20% of symptom-free relative of patients with dilated cardiomyopathy (DCM) from England and Italy. The role of autoimmunity may vary in DCM patients from Poland due to ethnic differences in genetic susceptibility to autoimmune disease. METHODS: We assessed the frequency of the organ-specific cardiac autoantibodies in 162 symptom-free relatives of DCM patients [85 male, mean (SD) age 27 (18) years] and 80 control subjects from Poland. Familial DCM (> 1 affected member) was present in 4 families, nonfamilial DCM in the remaining 24 pedigrees. We performed antibody screening and noninvasive cardiological assessment in the whole group. RESULTS: The frequency of cardiac-specific autoantibodies was higher among patients with documented DCM (probands and relatives) (50%) and their symptom-free relatives (38%) than in unrelated normal subjects (10%; p = 0.0001). In 24 (86%) of the pedigrees studied, autoantibodies were found in the proband and/or in at least one family member and tended to be more common in familial than in nonfamilial DCM (50 vs. 35%, p = NS). Echocardiographic indices of left ventricular size and function were similar in relatives with and without detectable antibodies. CONCLUSIONS: The presence of cardiac-specific autoantibodies in symptom-free relatives of DCM patients provides evidence for autoimmunity in the majority (86%) of our pedigrees, including both familial and nonfamilial forms of DCM.

Determinants of prognosis in nonischemic dilated cardiomyopathy.

BACKGROUND: Dilated cardiomyopathy, a heart muscle disease of unknown cause, is characterized by high mortality and is a major cause of cardiac transplantation. It has become, therefore, increasingly important to identify patients at higher risk. The aim of this study was to assess which of the data obtained at the time of diagnosis are the best predictors of survival. METHODS AND RESULTS: One hundred forty-four patients with dilated cardiomyopathy (118 men; mean age, 39 years) were assessed clinically, noninvasively, and hemodynamically. The effect of variables derived from the evaluation on outcome (death or heart transplantation) was examined. During a mean follow-up time of 4.1 years, 68 patients (47%) died and 9 (6%) underwent heart transplantation. The 1-, 2-, and 5-year transplant-free survival rate was 79, 69, and 44%, respectively. Cox multivariate regression analysis identified three variables as independent predictors of outcome: (1) pulmonary artery systolic pressure, P = .0001; (2) left ventricular ejection fraction, P = .0013; and (3) left ventricular end-diastolic dimension, P = .007. The prognostic index was constructed from regression coefficients and parameters significant in the Cox model. The minimal prognostic index in the study group was 1.4 and the maximal was 6.0 with a corresponding 1-year survival of 98 and 18%, respectively. The validity of the prognostic index was tested in the consecutive group of 81 patients, who were followed for a mean 2.3 years. The prognostic index of the poor outcome group differed significantly from that in survivors (3.7 vs 2.9, respectively, P < .01). The sensitivity and specificity of model predictions were 68 and 52%, respectively. CONCLUSIONS: The severity of pulmonary hypertension and left ventricular dysfunction provides an independent insight into the prognosis of patients with dilated cardiomyopathy. The prognostic index is useful when assessing prognosis and may be helpful in the timing of heart transplantation.

Left ventricular enlargement is common in relatives of patients with dilated cardiomyopathy.

Idiopathic dilated cardiomyopathy is characterized by dilation and impaired contractility of one or both ventricles. Long-term prognosis is poor. Early diagnosis has the potential for substantial reduction of morbidity and mortality. Recent studies, based on echocardiographic assessment of relatives of the patients have shown that familial dilated cardiomyopathy is relatively common. The authors studied 215 relatives (mean age, 27 years; 111 male) of 38 index patients with idiopathic dilated cardiomyopathy by clinical examination, electrocardiography, and two-dimensional, M-mode and Doppler echocardiography. Seven relatives (3%) from six families were shown to have dilated cardiomyopathy. Thus, 6 of the 38 index patients (16%) had familial disease. Furthermore, left ventricular enlargement either during diastole or systole was found in 66 of 174 healthy relatives (38%). This is significantly more frequent than in our normal control population of 100 unrelated subjects studied in the same way (18%; P < .0001). These 66 relatives with left ventricular enlargement belonged to 27 of the 38 examined families (71%). Dilated cardiomyopathy was found to be familial in 16% of patients. Of the relatives examined, 41% had left ventricular abnormalities. These findings provide further evidence for a genetic background of dilated cardiomyopathy. Relatives with left ventricular enlargement may have an early stage and/or latent form of the disease.

Organ-specific cardiac autoantibodies in dilated cardiomyopathy. Frequency and clinical correlates in Polish patients.

Organ- and disease-specific cardiac autoantibodies are found in a third of dilated cardiomyopathy patients from the U.K. and Italy and represent markers of autoimmune involvement. The role of autoimmunity may vary in dilated cardiomyopathy patients from different countries due to differences in genetic susceptibility to autoimmune diseases. The aim of this study was to assess the frequency of organ-specific cardiac autoantibodies detected by immunofluorescence in a consecutive series of patients with dilated cardiomyopathy and in disease and normal control subjects from Poland. The study groups included 79 patients with idiopathic (WHO criteria) dilated cardiomyopathy, 55 patients with other cardiac disease and 60 normal subjects. Cardiac antibody tests were performed by indirect immunofluorescence on human heart; skeletal muscle was used to identify cross-reacting antibodies. The frequency of organ-specific cardiac autoantibodies was higher in patients with dilated cardiomyopathy (21/79, 27%) than in controls with other cardiac disease (1/55, 2% P < 0.001) or in normal subjects (7/60, 12% P < 0.02). Conversely, cross-reactive antibodies were detected in similar proportions in patients with dilated cardiomyopathy (5/79, 6%), disease controls (7/55, 13%) and normal subjects (6/60, 10%, P = ns). The organ-specific antibody was more common in patients with dilated cardiomyopathy with insidious onset of disease (17/34, 50%) compared to those who did not exhibit this feature (4/45, 9%, P < 0.0001). Organ- and disease-specific cardiac autoantibodies were found in 27% of Polish patients with dilated cardiomyopathy at diagnosis; this is evidence for autoimmune involvement in a subset of patients from our country, as seen in a previously reported series of Western European origin. The association of antibody status with insidious onset of symptoms is in keeping with the long latency period observed in other autoimmune disorders.

Long term follow up after percutaneous mitral commissurotomy with the Inoue balloon-incidence of restenosis.

UNLABELLED: The long term outcome of 300 consecutive patients following percutaneous mitral commissurotomy (PMC) with the Inoue balloon was analyzed with regard to the incidence of restenosis. There were 256 females and 44 males (mean age 44.4 +/- 9.9 years, range 18-69 years), 52 had previous surgical commissurotomy, 96 were in atrial fibrillation, and 16 had a history of embolism. PCM was carried out with a success rate of 84% (no significant mitral regurgitation and mitral valve area (MVA) > 1.5 cm2). Two hundred and seventy patients were available for clinical and serial echocardiographic studies at six months, 12 months and once a year thereafter (18 patients operated on for mitral regurgitation less than six months after PMC, three patients lost to follow up, nine patients refused to return). MVA increased with PMC from 1.18 cm2 +/- 0.3 to 2.0 +/- 0.3 cm2 and then decreased to 1.8 +/- 0.3 at a mean follow up of 24.0 +/- 13.5 months (range 6-55). Echocardiographic restenosis (RS) (MVA at follow up < 1.5 cm2 with a 50% loss of the initial gain) was found in 38 patients (14%). Twenty-five (66%) of them remained in NYHA class I or II. Restenosis free survival according to the Kaplan-Mayer curve was 93%, 86%, 77% and 73% at 12,24,36 and 55 months respectively. None of the 24 clinical, hemodynamic, echocardiographic or procedural variables used on the Cox proportional hazard regression analysis identified predictors of restenosis free survival. CONCLUSIONS: The overall incidence of echocardiographic restenosis post PMC is low (12.6%) in patients followed for a mean period of two years and often occurs without worsened clinical symptoms. It may be difficult to define clinical, echocardiographic or procedural factors as significant predictors of restenosis free survival.

[Familial dilated cardiomyopathy with autosomal mode of inheritance. Case report]. / Kardiomiopatia rozstrzeniowa z dominujacym autosomalnym sposobem dziedziczenia. Opis rodziny.

Familial occurrence of dilated cardiomyopathy is estimated by 2-20%. We present a family with dilated cardiomyopathy inherited in an autosomal dominant way. We examined 9 members of the family, most of them are asymptomatic.