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BACKGROUND: Persistent pulmonary hypertension of the newborn (PPHN) is a rare syndrome with a severe prognosis, in which a prompt diagnosis can be life-saving. The aim of our study was to verify its prevalence in a neonatal population, define clinical and echocardiographic criteria for the diagnosis of PPHN and discuss therapeutic choices. METHODS: The following clinical and echocardiographic criteria for the diagnosis of PPHN were defined: 1. cyanosis and hypoxemia non-responsive to O2 therapy; 2. right to left shunt at an atrial or ductal level. All neonates fulfilling these criteria referred to the neonatal units of seven pediatric or general hospitals over a two-year period were enrolled. RESULTS: From January 1995 to December 1996, thirty neonates with PPHN (8%) were observed. Birth was pre-term in 5 out of 30. Ten (33%) had experienced chronic and/or fetal asphyxia (FA). Death occurred in 7 (22%), four of whom with FA. Echocardiography showed tricuspid insufficiency in 18 (60%); mean pulmonary systolic pressure was 67 +/- 16 mmHg (range 41-95). In 23 surviving neonates, normalization of clinical and echocardiographic parameters occurred in 8 +/- 5 days. THERAPY: In most neonates, vasodilators (tolazoline, prostacyclin) and/or nitric oxide were employed. CONCLUSIONS: PPHN is confirmed to be a rare pathological condition; prognosis is severe, particularly in neonates with FA. Echocardiography is a reliable non-invasive method for a prompt diagnosis and follow-up. Subsequent studies are needed to assess therapeutic choices.

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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.

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Patients who have undergone surgical repair of congenital heart diseases are usually not allowed to participate in competitive sports. In the present study we report our long-term experience with 9 male athletes aged 17 to 23 years who participate in competitive sports after undergoing surgical repair of ostium secundum atrial septal defect at a median age of 9 years; six of them play football and three of them volleyball. Competitive sport activities began 1 to 5 years after surgical repair. The mean duration of follow-up is 88 +/- 26 months. Sport fitness was granted on the basis of the following criteria: 1) a normal physical examination; 2) a normal working capacity on exercise test; 3) no arrhythmias on exercise test and Holter monitoring, recorded also during sport activities; 4) a normal M-mode and two-dimensional echocardiography, including the normalization of right ventricular size; the persistence of an abnormal ventricular septal motion did not exclude sport fitness. Recently we also performed Doppler and color Doppler echocardiography and gated equilibrium radionuclide angiography at rest and during exercise. We studied left ventricular diastolic filling through the pulsed wave Doppler evaluation of transmitral flow and measured cardiac output by continuous wave Doppler echocardiography during exercise test in the supine position. We also performed exercise test and M-mode, two-dimensional, Doppler and color Doppler echocardiography in a control group made up of 15 athletes (10 football players and 5 volleyball players). The exercise duration at graded treadmill exercise test (according to the Carù protocol), the maximal heart rate and the maximal systolic blood pressure were, respectively, 12.9 +/- 0.8 min, 192 +/- 10 beats/min and 198 +/- 12 mmHg. Left ventricular end-diastolic dimension, mass and ejection fraction (single-plane area-length method) were 50.3 +/- 2.8 mm, 210 +/- 38 g and 65 +/- 6%. M-mode right ventricular diastolic dimension was 23.4 +/- 1.6 mm; the right ventricular maximal diastolic diameter and area obtained on two-dimensional echocardiography from the apical four chamber view were 44.1 +/- 3.6 mm and 25 +/- 3.8 cm2 respectively. The evaluation of transmitral flow showed the following data: E velocity 77 +/- 12 cm/sec, A velocity 45 +/- 6 cm/sec, E/A ratio 1.7 +/- 0.3, the isovolumic-relaxation period 72 +/- 8 m/sec and the deceleration half-time of the early rapid filling 71 +/- 10 m/sec. A trivial tricuspid regurgitation was detected in 6 subjects; the peak velocity of the regurgitant jet was less than 2.1 m/sec.(ABSTRACT TRUNCATED AT 400 WORDS)

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Thirty-six infants under one year of age underwent surgical repair of coarctation of the aorta between 1968 and 1983 in our institution. Coarctation was isolated or associated to a patent ductus arteriosus in sixteen cases, while in twenty it was associated with significant intracardiac disease. Twenty-nine patients were operated on in the first three months of life and in twenty a severe heart failure was present before the operation. Nine patients (25%) died while in the hospital: all of them were less than three months of age and all but one were affected by major intracardiac anomalies and severe heart failure. Surgical repair was by subclavian flap aortoplasty in twenty-nine cases, resection with end-to-end anastomosis in three, patch aortoplasty in three and Blalock-Park anastomosis in one. Operative mortality was unaffected by the surgical technique. The surviving children were followed-up for 30 +/- 7 months; one late death occurred suddenly, two months after the repair of a ventricular septal defect. An arm/leg pressure gradient, indicative of recoarctation, was detected in five cases: only one had been repaired by the subclavian flap technique, while the others were the only survivors of the end-to-end anastomosis and patch aortoplasty group. Subclavian flap aortoplasty is suggested as the operation of choice for coarctation of the aorta in the first year of life.

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