Williams-Beuren syndrome. Long-term results of surgical treatments in six patients.

To settle long-term outcome after surgery for supravalvular aortic stenosis in the Williams-Beuren syndrome, we reviewed the records of 6 patients who had repair of the localized form (n = 5) or diffuse form (n = 1) at our Institution from 1965 to 1971. Four patients were females and 2 males, ages at operation ranged from 9 to 16 years (mean = 13 +/- 2.37 years). In all the patients was present the typical elfin facies with mental retardation and reduced I.Q. Preoperative omeral pressure was different between left and right arm (89 +/- 7/67 +/- 8 vs 105 +/- 8/77 +/- 4). Chest X-ray showed and enlargement of the cardia silhouette in all the patients. Cardiac catheterization, performed in all the patients, allowed diagnosis of supravalvular aortic stenosis and, in one case of subaortic stenosis associated. Intraoperatively a coronary tree enlargement was found in all cases with particular involvement of the right coronary in two patients. The mean diameter of the ascending aorta was 5.67 +/- 1.97 mm but the smallest (3 mm) was in the diffuse group. In group with localized stenosis the aortic root was enlarged with a teardrop patch in Dacron (n = 4) or a simple transverse suture after a longitudinal incision (n = 1). A pantaloon-shaped patch was necessary in the diffuse form case. There were no operative deaths and all the patients were discharged from the hospital after 2 weeks. A clinical follow-up was possible in all the patients (10%) extended from 25 to 30 years (mean = 27.7 +/- 2.19 years); there were no late deaths and at presents time the mean age of the patient is 40 +/- 3 years. All patients were in functional class I or II. There was no significant difference between patients with a teardrop-shaped or pantaloon-shaped patch in terms of late gradient, survival, or aortic insufficiency studied by Echocardiography and color-Doppler. Of six patients two are living with parents or relatives but four are in a farm-college for disable people working and having some responsibility. We conclude that surgery for the correction of supravalvular aortic stenosis in Williams-Beuren syndrome is mandatory and both the procedures with patch techniques provide excellent long-term results of gradients and aortic valve competence. Moreover the patients after the operation can have a normal activity with a satisfactory style and expectation of life.

Effect of pleurotomy on pulmonary function after coronary artery bypass grafting with internal mammary artery.

Coronary artery by-pass grafting with internal mammary artery (IMA) has become the graft conduit of choice, due to improved survival and its long term patency rate. However, some studies have shown that, in comparison with saphenous vein grafts, after IMA grafting, there is increased postoperative impairment of pulmonary function, possibly due to the frequent performance of pleurotomy. In 57 consecutive patients, admitted for elective CABG with IMA, we prospectively evaluated the early (2nd and 6th day) postoperative chest X-ray complications and the late (2 months) respiratory function tests changes. Thirty-two patients had been subjected to pleurotomy (group 1) and 25 not (group 2). The incidence of pulmonary atelectasis and pleural effusion in 2nd and in 6th postoperative days was not different in the two groups: 22 vs. 19%, 74 vs. 52% in 2nd, and 29 vs. 19%, 48 vs. 38% in 6th postoperative day respectively. The incidence of elevated hemidiaphragm in 6th postoperative day was not different in the two groups (18.5 vs. 14%). Two months after surgery the mean values of spirometric tests were significantly lower than the preoperative values: VC from 88.5 +/- 1.26 to 80 +/- 1.65% of predicted, P < 0.001, FEV1 from 96.1 +/- 1.27 to 84.7 +/- 1.73% of predicted, P < 0.001, MEF50 from 84.9 +/- 3.14 to 69.2 +/- 3.18% of predicted, P < 0.001. No significant changes were detected in RV and in AaPO2.(ABSTRACT TRUNCATED AT 250 WORDS)

[Is coronary angioplasty less costly than aortocoronary bypass?]. / L'angioplastica coronarica è meno costosa del bypass aortocoronarico?

We retrospectively compared the cost-benefit ratio of coronary bypass grafting (CABG) and percutaneous coronary angioplasty (PTCA). Data were obtained on 40 CABG's and 40 PTCA's patients treated from 15/2/86 to 15/9/86. All patients had at last 1-year follow-up. PTCA and CABG groups had similar baseline clinical and angiographic criteria. We analyzed the following cost components: real estate, biomedical products, drugs, hospital charges, medical fees, cardiac and non-cardiac tests. Total cost of CABG exceeds that of PTCA by a factor of 1.64: 15,095,000 vs 9,201,346 Italian liras (ILit). Taking into account early and late medical and surgical complications, the factor decreases to 1.39: 15,746,500 vs 11,323,000 ILit. After 1 year of follow-up the factor decreases to 1.18: 16,613,500 vs 14,027,500 ILit. Our data show that the initial savings were reduced when complications and follow-up are considered, but PTCA's patients had shorter hospitalization, earlier return to work, lower psychosocial stress.

Low-dose dopamine during cardiopulmonary bypass in patients with renal dysfunction.

Thirty-six patients with preoperative renal dysfunction were studied to evaluate the effects of dopamine (D) and dopamine-nitroprusside (DN) on renal function during cardiopulmonary bypass (CPB). No differences from the control group (C) were found in creatinine clearance, fractional sodium excretion, osmolarity and free-water clearance. Sodium output/intake ratio during CPB was higher in group D than in groups C and DN (P less than 0.05); water output/intake ratio was higher in group D than in group C (P less than 0.05). Urine lysozime levels and alpha-glycosidase/creatinine ratios increased similarly in the three groups, suggesting ischemic tubular cell damage. No patients showed acute postoperative renal failure or a worsening of their renal dysfunction. The data suggest an increased water and sodium excretion during CPB with a dopamine infusion, possibly resulting from a renal vasodilator effect that was abolished by simultaneous nitroprusside administration.

Morphological comparison of primary tissue failure (PTF) in porcine mitral and aortic bioprostheses in the same patient.

Clinical and pathological studies have not clearly demonstrated whether primary tissue failure (PTF) in porcine bioprostheses occurs more often in the mitral than in the aortic position. We have studied morphological alterations in both positions in the same individual in 15 patients (14 mitroaortic and 1 mitroaortotricuspid) reoperated upon for PTF. Bioprostheses explanted were photographed, radiographed and observed in transmitted polarizing light. All lesions received a score on the basis of morphological criteria. The creep of the stent was measured. Calcification was slightly heavier and the degree of creep was significantly greater in the mitral position. Tears, infiltration and pannus growth did not differ between the two positions. According to our study, there is no conclusive demonstration that bioprostheses degenerate earlier and more extensively in the mitral than in the aortic position.

Results of total correction of tetralogy of Fallot performed in adults.

Today, total correction of tetralogy of Fallot is rarely performed in adults. In a 10-year period, 40 patients aged 20 to 67 years underwent intracardiac repair in our institution. Twenty-eight of them had had a palliative procedure 11 to 30 years earlier. Preoperatively, 23 patients were in New York Heart Association (NYHA) Functional Class II, 14 were in Class III, and 3 were in Class IV. Operative mortality was 2.5% (1/40). Follow-up ranged from 1 year to 11 years (average, 3 years). One patient died of a noncardiac cause 4 years after operation. Residual cardiac defects were observed in 4 patients. Postoperatively, 30 patients were in NYHA Functional Class I, 8 were in Class II, and 1 was in Class III. Major ventricular arrhythmias were recorded in 7 (35%) of 20 patients. Radionuclide angiography demonstrated impaired right ventricular function in 8 patients. Left ventricular impairment was present in 2. Total correction of tetralogy of Fallot can be performed safely in adults with low mortality and good functional improvement. The incidence of residual cardiac defects is low. The long-term importance of impaired ventricular function and arrhythmias remains to be ascertained.

Protection of the heart by nifedipine cardioplegia during coronary artery surgery. A clinical-haemodynamic evaluation.

This study was undertaken to evaluate the myocardial preservation obtained by adding a Ca++ channel blocker, nifedipine, to cold potassium cardioplegia (4 mcg/Kg/L) in 24 patients undergoing coronary artery surgery. They were randomly divided into a treated (N) and a control (C) group. Significant differences between the two groups were noted in the cardiac arrest time (p less than 0.001), in the mechanical recovery mode (p less than 0.01) and in the inotropic support needed (p less than 0.01). Cardiac index increased significantly in group N but decreased in group C (p less than 0.01). Peripheral delta P/delta t and endocardial viability ratio (EVR) decreased in both groups. Coronary sinus and serum CK and CK-MB release were significantly lower in the treated group. ECG ischaemic changes occurred in 8 patients in group C but only in 1 case in group N (p less than 0.001). Arrhythmias occurred in 3 cases in group C (p less than 0.05). The incidence of perioperative myocardial infarction was not significant (2 cases in group C). These data suggest that nifedipine can protect the myocardial cell from ischaemic injury without depressing myocardial contractility or AV conduction.

Carpentier-Edwards bioprosthesis: a 7-year follow-up in 361 patients.

Three hundred sixty-one patients undergoing heart valve replacement with Carpentier-Edwards bioprostheses (CE) were evaluated: 169 patients underwent replacement of the mitral valve (MVR), 124 of the aortic valve (AVR), one of the tricuspid valve (TVR) and 67 of 2 or more valves (mVR). A total of 420 CEs were implanted. Overall operative mortality was 4.7% (7.1 for MVR, 1.6 for AVR, 4.5 for mVR). Early complications included hemorrhage (5 cases), thromboembolism (one case), paraprosthetic leak (one case). Actuarial survival rates at 5 years were computed for overall deaths, cardiac deaths and prosthetic deaths. In the 3 subgroups these 3 rates were respectively 78.7 +/- 5.6%, 82.9 +/- 5.4%, 96.4 +/- 1.6% for MVR, 88.9 +/- 3.3%, 94.2 +/- 2.3%, 98.7 +/- 1.3% for AVR, and 82.3 +/- 5.9%, 88.3 +/- 5.1%, 98.5 +/- 1.5% for mVR. Actuarial freedom from prosthetic complications at 7 years was 59.6 +/- 7.5%, 78.1 +/- 4.1% and 61.9 +/- 8.0% for MVR, AVR and mVR patients. Thromboembolism-free rates were 87.2 +/- 3.9% and 99.2 +/- 0.8% for MVR and AVR, while the thromboembolic linearized rate was 2.8% per patient-year and instantaneous risk of thromboembolism was 4.1% at one and instantaneous risk of thromboembolism was 4.1% at one year for MVR. Anticoagulant-related hemorrhage linearized occurrence was 1.0%, 1.2% and 0.8% per patient-year among the 3 subgroups. Prosthetic endocarditis occurred in 12 patients with a linearized rate of 1.1% per patient-year. Eleven of these patients were treated successfully. Three episodes of primary tissue failure were recorded (0.2% linearized rate per year, instantaneous risk 3.5% at the sixth year).(ABSTRACT TRUNCATED AT 250 WORDS)

Diltiazem cold cardioplegia in coronary artery surgery: effects on myocardial function and ischemia.

This study was undertaken to evaluate the myocardial preservation obtained by adding a calcium channel blocker, diltiazem (200 mcg/kg of body weight) to cold potassium cardioplegia in 24 patients with coronary artery disease who underwent myocardial revascularization, and were randomly divided into a treated Group D and a Control Group C. No significant differences were noted between the groups in cardiac electrical arrest and recovery time, recovery rhythm, mechanical function recovery, inotropic support, stroke index, or cardiac index. After cardiopulmonary bypass, the stroke index decreased by 13.1% in Group D and by 20.7% in Group C; cardiac index increased in both--18.6% and 14.0%, respectively--but the results were possibly from compensatory heart rate increases. Peripheral deltaP/deltat and endocardial viability ratios decreased in both groups. Coronary sinus enzymes and serum CK were slightly lower in the treated group. Serum CK-MB, however, was significantly higher in the Control Group. Electrocardiographic ischemic changes occurred in four patients, all in Group C (p =.046). Hyperkinetic arrhythmias occurred in five of the controls, but in none of the treated patients (p =.018). One perioperative myocardial infarction was diagnosed in the Control Group. These data suggest that diltiazem has no negative side effects on hemodynamics or cardiac rhythm and does not reduce impairment of myocardial function due to ischemia, but it does decrease the incidence of ischemic lesions in patients undergoing coronary artery bypass procedures.

Postoperative histological diagnosis in two cases of unsuspected bioprosthetic endocarditis.

Two cases of preoperatively unsuspected porcine bioprosthetic endocarditis are presented. Both cases, thought to be a sterile thrombosis and a primary tissue failure respectively, were correctly diagnosed only with histological examination. Cultures and histological examination of the explanted tissue are necessary for a definite diagnosis in every case of prosthetic malfunction.

[Aortic coarctation and aneurysms of the ascending aorta]. / Coartazione aortica ed aneurismi dell'aorta ascendente.

Dissection and rupture of the aorta account for 20% of death in the natural history of aortic coarctation. We describe here in four patients in whom an ascending aortic aneurysm was associated with aortic coarctation. Three patients had aortic valve incompetence. In two cases there was a dissection. This had not been recognized preoperatively in one, in whom the intimal tear was small; in the other patient, with dissection and shock, the associated mild coarctation was demonstrated only at autopsy. Surgical treatment of patients with aortic coarctation and associated aortic aneurysm must include resection of both the stenotic isthmus and dilated section of the aorta, because of a documented poor prognosis. Furtherly, when evaluating patients with aortic dissection a coexisting coarctation although infrequent must be ruled out. If such an association is identified in emergency cases the dissected aorta should be repaired first, employing a suitable technique (double arterial cannulation, above and below the isthmus) in order to ensure adequate perfusion. When there is no acute dissection, repair of the coarctation should be undertaken first.

Coronary artery bypass grafting for unstable angina. Risk factors of operative mortality.

In order to identify factors affecting early mortality in patients undergoing CABG for unstable angina, several risk factors have been analysed in a group of 120 patients. Systemic hypertension and left ventricular impairment were shown to be significant risk factors (Systemic hypertension, P less than 0.01; EF less than or equal to 0.35, P less than 0.01; LVEDP greater than or equal to 20 mmHg, P less than 0.025). Overall mortality rate was 5% (6/120). No influence could be recognized for age, sex, previous MI, emergency surgery, extension of coronary disease, completeness of revascularization and mode of onset of symptoms.