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.

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.

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

Dilated cardiomyopathy in children: clinical course and prognosis.

The clinical profile of 19 patients with dilated cardiomyopathy ages 2-18 years (mean 13.4 +/- 4 years) was reviewed to detect any factors that might be predictive for their survival. Follow-up range from 5 to 105 months (mean 39 +/- 33 months). Routine treatment consisted of digitalis and diuretics: 14 patients received antiarrhythmics, 6 received vasodilators, and 12 were managed with immunosuppression. There were 12 survivors and 7 nonsurvivors: The 1-year mortality was 21.2% and the 2-year mortality 35.8%. All deaths were within first 2 years. Of the 12 patients who survived 2 years, a significant improvement was noticed in 9. In 3 patients tachycardia-induced cardiomyopathy was diagnosed, and abolition of supraventricular tachycardia was followed by improvement and regression of cardiomegaly. Endomyocardial biopsy was performed in 16 patients. Four with a histologic diagnosis of active myocarditis survived, and in 3 of them a considerable improvement was noticed. Of the 12 patients with nonspecific histologic findings, 6 died (p < 0.05). There were no significant differences between survivors and nonsurvivors for any of the following parameters: incidence of severe heart failure (NYHA class III-IV) and severe ventricular arrhythmias (Lown class III-V), relative heart volume, echocardiographic left ventricular diastolic diameter and shortening fraction, and the hemodynamic parameters of cardiac index, left ventricular ejection fraction, left ventricular end-diastolic pressure, and left ventricular end-diastolic volume index.

[Aortic-left ventricular tunnel--description of two cases]. / Tunel aortalno-lewokomorowy--opis dwóch przypadków.

This is a very rare congenital anomaly, which clinically imitates aortic insufficiency and therefore should be kept in mind by the cardiologist. Only an early precise diagnosis and proper surgical treatment will result in a good long-term prognosis. We have diagnosed and treated two patients, four and five years old, referred to us with a diagnosis of aortic insufficiency. Our final assessment was based on an obligatory cine-angio study, since a certain percentage of patients with this anomaly have concomitant lesions in the cardio-vascular system, which should be precisely detected. A brief differential diagnosis and discussion concerning different surgical techniques are presented. In our two cases obliteration of the tunnel using a Dacron patch inserted vertically in the tunnel with closure of both ostia was performed. This technique seems to be superior compared to the previous one in terms of preventing the progressive destruction of the aortic valve. All the authors agree that early surgical treatment is indicated before the left ventricle becomes dilated and the aortic valve significantly damaged. Two years post surgery our patients are in very good condition with: no signs and heart failure, a gradual diminution of the left ventricular cavity and satisfactory function of the aortic valve.

[Percutaneous pulmonary valvuloplasty in 135 patients]. / Przezskórna walwuloplastyka plucna u 135 chorych.

We present our 7 years experience with 135 pts aged 1.4-44 years (mean 10-yrs) in whom percutaneous balloon valvuloplasty (BPV) for congenital pulmonary stenosis was attempted. In 4 pts we failed to place the balloon at valvar level, in another four BPV was repeated so, 135 procedures in 131 pts were performed. Balloon diameter/pulmonary anulus diameter ratio (BD/PD) ranged from 0.9 to 1.85 (mean 1.37). In 10 cases double balloon technique was used. Immediate results: in the whole group of 131 pts the right ventricular-pulmonary artery gradient (RV-PAG) was reduced from 74 +/- 42 to 34 +/- 34 mm Hg, and right ventricular systolic pressure (RVSP) decreased from 92 +/- 41 to 54 +/- 34 mm Hg just after BPV. 86 pts (65.6%)--group I, had good immediate result of BPV (RVSP less than 50 mm Hg). 4 of them had dysplastic pulmonary valves (DPVs). RV-PAG in 82 pts with normal valves decreased from 53 +/- 25 to 15 +/- 7 mm Hg right after BPV. In 45 pts (34.4%)--group II, immediate result of valvuloplasty was recognised as unsatisfactory (RVSP greater than or equal to 50 mm Hg): in 4 of them BD/PD was smaller or equal 1.2 (subgroup IIa); 34 others had significant subpulmonary obstruction (SPO) that appeared or increased after BPV (subgroup IIb); and in remaining 7, DPVs were noticed (subgroup II c).(ABSTRACT TRUNCATED AT 250 WORDS)

[Dilated cardiomyopathy in children and adolescents. Diagnostic problems, clinical course and prognosis]. / Kardiomiopatia rozstrzeniowa u dzieci i mlodziezy. Problemy diagnostyczne, przebieg kliniczny i rokowania.

UNLABELLED: The clinical profile of 19 patients with dilated cardiomyopathy from 2 to 18 years old (mean age 13.4 +/- 4 years) was reviewed to detect any factors that might be predictive for their survival. Follow-up study ranged from 5 to 105 months (mean 39 +/- 33 months). All patients received digitalis + diuretics, 12 were managed with immunosuppression, 16 with antiarrhythmics. There were 12 survivors and 7 nonsurvivors: the 1-year mortality was 21.2%, the 2-years mortality was 35.8%. All deaths were within first 2 years. In 12 patients who survived 2 years, significant improvement was noticed in 9 cases. Endomyocardial biopsy was performed in 16 patients. Four of them with histological diagnosis of myocarditis survived and in 3 of them a considerable improvement was noticed. Half of 12 patients with nonspecific histological findings died (p less than 0.05). There was no significant difference between survivors and nonsurvivors in all following parameters: the incidence of severe heart failure (NYHA class III-IV) and severe ventricular arrhythmias (Lown III-IV), relative heart volume, echocardiographic LVDD, haemodynamic parameters--CI, LVEF, LVEDP, LVEDVI. CONCLUSIONS: Clinical, electrocardiographic, echocardiographic and haemodynamic data are nonpredictive for survival. The most dangerous period are the first two years of illness. In long term, improvement was noticed in half of patients.

[Late results of immunosuppressive therapy in patients with heart failure and biopsy proven myocarditis]. / Odlegle wyniki leczenia immunosupresyjnego u chorych z niewydolnoscia serca i bioptycznie rozpoznanym zapaleniem miesnia sercowego.

Late results of 6-month immunosuppressive therapy (prednisone from 1.5 mg/kg b.w. and azathioprine 2 mg/kg b.w.) simultaneously performed with a conventional treatment were analyzed in 20 patients with heart failure of unknown origin and bioptic diagnosed myocarditis. Average patients' age was 33.8 +/- 10.7 years, mean disease duration--7.8 month, mean left ventricular ejection fraction--25.9 +/- 8.9%. Follow up period was at least 24 months in all patients. 1 patient died before the end of therapy. After 6-month immunosuppressive therapy improvement was stated in 10 patients (50%)--group A, stable disease course in 3 (15%)--group B and deterioration in remaining 6 (30%)--group C. After the next 18 month conventional therapy as many as in 8 of 10 group A patients deterioration was observed, further improvement (EF increase from 17% to 43%) in 1 female patient and a stable disease course in the another female. Of 3 group B patients in 1 further improvement was observed and a stable course in 2 remaining. Of 6 group C patients 4 died, 1 underwent cardiac transplantation, 1 female patient is still alive, but does not put herself to control examinations. Early improvement after 6-month immunosuppressive treatment does not prejudge the later prognosis.