Cardiac findings in 31 patients with Noonan's syndrome.

OBJECTIVE: To evaluate cardiac findings in 31 Noonan syndrome patients. METHODS: Thirty-one (18 males and 13 females)patients from 26 families affected with Noonan's syndrome were evaluated from the cardiac point of view with electrocardiography and Doppler echocardiography. RESULTS: Twenty patients had some type of cardiac abnormality. The most frequent was pulmonary valve stenosis followed by hypertrophic myocardiopathy, commonly associated with valve defects. Upper deviation of the QRS axis was observed in 80% of these patients. CONCLUSION: In view of the high frequency and diversity of cardiac abnormalities present in Noonan syndrome, cardiac evaluation with electrocardiography and echocardiography should be performed in all patients diagnostically suspected of having this disease.

Endocardite bacteriana como complicaçäo de sepse neonatal: relato de caso / Bacterial endocarditis as a complication of neonatal sepsis: a case report

Os autores relatam um paciente com 11 dias de vida, internado em Unidade de Terapia Intensiva Neonatal devido a múltiplas malformações congênitas, apresentando sepse e endocardite bacteriana. Entre os fatores de risco para endocardite foram destacados o cateterismo venoso central, hemocultura com crescimento de Staphylococcus aureus e ventilação mecânica. O diagnóstico foi realizado no 61§ dia de internação devido a presença de febre persistente e aparecimento de sopro cardíaco sistólico. O ecocardiograma mostrou trombo em átrio direito, medindo 1,9 x 0,7mm sendo realizada antibioticoterapia e ressecção cirúrgica, com melhora clínica. No 125§ dia de internação ocorreu óbito devido à sepse e abscesso cerebral. Na necrópsia não foram observados malformações cardíacas. Os autores concluem ser de grande importância o conhecimento das complicações potenciais das técnicas invasivas utilizadas em recém-nascidos criticamente doentes. A suspeita clínica de endocardite deve ser realizada em todos os neonatos com sepse, internados em Unidade de Terapia Intensiva Neonatal por tempo prolongado.

[Bacterial endocarditis as a complication of neonatal sepsis: case report]. / Endocardite bacteriana como complicação de sepse neonatal--relato de caso.

The authors reported on a 11 day-old child, admitted in Neonatal Intensive Care Unit for multiple congenital malformations, who had sepsis and bacterial endocarditis. Among the risk factors for endocarditis were outstanding: the central venous catheterism, hemoculture with growth of Staphylococcus aureus and mechanical ventilation. The diagnosis was made in the 61st day after admission owing to the presence of persistent fever and appearance of systolic murmur. The echocardiogram revealed a thrombus in the right atrium measuring 1.9 x 0.7 mm. Antibiotic therapy and surgical resection being performed, with clinical improvement. On the 125st day after admission the patient died owing sepsis and cerebral abscess. At necropsy, heart malformations were not observed. The authors concluded to be very important the knowledge of the potential risks of invasive procedures currently used to care for critically ill newborns. The clinical suspicion of endocarditis should be considered in all neonates with sepsis and receiving intensive care for long time.