Introduction: Only few data are available in literature regarding the long term outcome of the newborns with isolated congenital complete heart block (CHB). The aim of the study was to describe neonatal morbidity, incidence of minor heart defects, mortality and the risks of poor outcome in the patients with CHB diagnosed in utero or as a newborn.Patients and methods: Since 1950, CHB whithout structural abnormality has been diagnosed in 152 children aged between 0-15 years in five tertiary centers in Finland. Diagnosis was made in utero or postnatally in 91 children with a median gestational age of 29.3 weeks. Maternal connective tissue disease was evident in 90% of the cases. At birth the median gestational age was 37.1 weeks (range 29–41) and the median birthweight 2969 g (range 905–4370 g). Sixty infants of 91 (66%) were girls and 7 of 91 (8%) were twins. Mean follow-up time was 10.6 years (range 0–47.5 years).Results: Fetal heart rate at diagnosis was 57 beats/min (median), and after birth 56 beats/min. Delivery route was cesarean section in 57% of cases. Insidence of neonatal morbidity was 58%; hydrops 27%, cardiac failure 46%, symptoms of neonatal lupus 18%, symptomatic PDA 16%, RDS 8%, NEC 7%, 1VH 6% and pulmonary hypertension 4%. There were 6 perinatal deaths (7%; one in utero and 5 postnatally). Pacing as a newborn was indicated in 48 of 90 cases (53%); 36 received pacemaker (PM) at older ages.

This unique case is of a man, followed clinically since infancy, who had a ventricular septal defect which closed spontaneously, a small arterial duct, and a minor degree of aortic coarctation, all without obvious symptoms. He later developed progressive cardiac failure which was attributed to some obscure form of congenital cardiomyopathy. He died at the age of 45 years. Necropsy showed a grossly abnormal arrangement of ventricular myocardial fascicles and bands, with absence of the papillary muscles causing tricuspid and mitral regurgitation. The various malformations are considered to be a gross example of a diffuse congenital cardiovascular disease complex, to the best of our knowledge previously undescribed.

The report by Danetz, Donofrio and Embrey1 provides an interesting long term follow-up on the first patient diagnosed by me with what is now known as Noonan syndrome. In 1960, when this patient was seen, cardiac ultrasound was unavailable and left-sided cardiac catheterization uncommon. He had severe pulmonary stenosis, and did well following surgery. Unfortunately, his follow-up was not adequate and left-sided obstruction was not recognized until he became symptomatic some thirty years later. Early reports stressed the dysplastic pulmonary valve, with pulmonary stenosis as a common finding.2 By 1972, the report of Ehlers et al.3 brought attention to the left-sided involvement in the Turner phenotype.

In recent years, rheumatic fever has been declining in the industrialized countries, and has became a rare disease. In developing areas, nonetheless, the sequels of its cardiac involvement have important implications from the stance of public health, and this disease is still the main cause of heart disease in children and young adults. From a historical perspective, the long-term prevention and the control of socioeconomic problems have been proven to be effective, and it is these measures which have contributed to the decline of the disease in developed countries. But, due to its present high prevalence around the world, and the potential for his resurgence, this disease remains an unsolved problem. Furthermore, the complete understanding of its pathophysiology remains a challenge. In this presentation, we will discuss our experience with epidemiological, clinical and prophylatic aspects of this enigmatic disease, and the strategies we have developed to study and control heumatic fever in Minas Gerais, Brazil.