[Postoperative follow-up of type A aortic dissectionexperience with 67 patients]. / Follow-up postoperatorio della dissezione aortica di tipo A; esperienza su 67 pazienti.

BACKGROUND: The surgical treatment of type A aortic dissection is usually palliative and most surviving patients remain at considerable risk to develop late postoperative complications; consequently, there is the need for careful long-term follow-up. The present study reports on our experience in the postoperative follow-up of a consecutive series of patients with type A aortic dissection. METHODS: Between January 1986 and December 1996, 89 patients underwent emergency surgery for type A acute aortic dissection; the overall hospital mortality rate was 22% (20/89). This study includes the 69 hospital survivors (49 men and 20 women). Forty-six patients had ascending aortic graft replacement, 13 patients underwent replacement of aortic valve and ascending aorta by a composite graft. The surgical repair was extended to the aortic arch in 5 patients. All patients were serially evaluated by clinical examination and imaging techniques (transthoracic echocardiography in all patients, magnetic resonance imaging in 40, transesophageal echocardiography in 33 and computed tomography in 25). Follow-up was complete in 97% of patients (two patients were lost to follow-up and excluded from the study) and extended to a maximum of 152 months (mean 74 +/- 39 months). The postoperative quality of life was assessed by a questionnaire in 51 current survivors. Risk factors for cardiovascular death, reoperation and poor quality of life were investigated with univariate and multivariate analysis. RESULTS: During the follow-up period 15 patients (22%) died; in 13 cases death was due to cardiovascular causes and in 6 of them it was related to aortic disease. The Kaplan-Meier survival was 92 +/- 3%, 87 +/- 5%, 78 +/- 6% and 70 +/- 8% at 2, 4, 6 and 8 years, respectively. A persistent aortic dissection was demonstrated in 50 patients (75%) and 42 of them showed the presence of flow in the false lumen. A dilatation of one or more aortic segments was found in 59 patients (88%), with a diameter > or = 50 mm in 17 and > or = 60 mm in 8. In 30 patients who underwent transesophageal echocardiography the relation between aortic dimensions and flow pattern in the false lumen was examined; the presence of aneurysmal dilatation with a diameter > or = 50 mm was significantly correlated with a "high flow" pattern. Ten patients (15%) underwent reoperation from 13 to 83 months postoperatively. Reoperation was indicated for: sinus of Valsalva aneurysm and severe aortic regurgitation (2 patients), severe aortic regurgitation (2 patients), aneurysm of the arch (1 patient), thoracoabdominal aneurysm (1 patient), periprosthetic pseudoaneurysm (4 patients). The hospital mortality rate was 20% (2 patients). Sixty-two% of current survivors are asymptomatic; 30 patients returned to their predissection status. Quality of live is judged "good" by 23 patients, "fairly good" by 21 patients and "poor" by 7 patients. No significant independent risk factor for cardiovascular death, reoperation and poor quality of life was identified. CONCLUSIONS: The long-term prognosis after surgical treatment of type A aortic dissection is not satisfactory because of a significant risk of late complications. However, the results of our study can be judged fairly good, particularly if we consider the natural history of the disease.

[Congenital isolated complete atrioventricular block: long-term experience with 38 patients]. / Blocco atrioventricolare completo congenito isolato: esperienza a lungo termine in 38 pazienti.

BACKGROUND: The management of patients with isolated congenital complete heart block is controversial, and indications for cardiac pacing are not clearly defined. In the present study we report our long-term experience in the management of patients with this disease. METHODS: Thirty-eight consecutive patients aged 2 days to 28 years (mean age 10 years) were studied and followed up for 130 +/- 57 months (range 18 to 274). They underwent an extensive evaluation including history, physical examination, electrocardiography and echocardiography; thirty-seven patients had Holter monitoring and exercise test. Electrophysiologic study was performed in 24 patients. RESULTS: Twenty-two patients were asymptomatic, sixteen had symptoms as syncope or presyncope (7 patients), marked exercise intolerance (1 patient), presyncope and marked exercise intolerance (1 patient), heart failure (1 patient), mild dyspnea on exertion (6 patients). Electrocardiograms showed a narrow QRS in all patients. Holter monitoring showed a marked bradycardia (awake heart rate < or = 55 beats/min in infants, < or = 40 beats/min in children and adults) in 15 patients and junctional pauses of > 3 seconds in 9 of them. The exercise test showed a markedly reduced exercise tolerance in 2 patients and exercise-induced complex ventricular arrhythmias in 3 patients. Echocardiography showed a structurally normal heart and a normal left ventricular function in all patients. The electrophysiologic study always showed a suprahisian site of block. Twenty patients (53%) underwent cardiac pacing at a median age of 14 +/- 10 years and were followed up for 110 +/- 59 months (range 18 to 253) after pacing; prophylactic pacing was performed in 10 patients. Indications for cardiac pacing were: syncope or presyncope (7 patients), presyncope and marked exercise intolerance (1 patient), neonatal heart failure (1 patient), marked exercise intolerance (1 patient), neonatal marked bradycardia (2 patients), marked bradycardia with junctional pauses of > 3 seconds and/or complex ventricular arrhythmias (7 patients), complex ventricular arrhythmias (1 patient). No death occurred during the follow-up. In 9 of 20 patients who had cardiac pacing, indication for this procedure appeared during the follow-up (development of symptoms, marked bradycardia and/or complex ventricular arrhythmias). Complications of pacing were infrequent (9 complications in 7 patients) and mainly occurred in the first years of our experience. At present 12 patients have an atrioventricular sequential pacing and 8 have a rate-responsive ventricular one. All patients who had pacing showed an improvement of exercise tolerance; 11 of them underwent exercise test after pacing which showed a significant increase in exercise duration (from 11.1 +/- 1.9 to 15.3 +/- 1.1 min, p < 0.01). In the 3 patients with complex ventricular arrhythmias we observed their suppression after atrioventricular sequential pacing. At present 35 patients are asymptomatic and 3 have mild dyspnea on exertion. CONCLUSIONS: Our results confirm that patients with isolated congenital complete heart block often have symptoms and/or signs of electric instability without symptoms. Cardiac pacing relieves symptoms and improves working capacity. Although in this study we are not able to draw conclusions on the therapeutic value of prophylactic pacing, our favourable follow-up results suggest that this therapeutic approach may prevent complications.