Cerebral arterial gas embolism in a patient with hypoplastic left heart syndrome treated with emergent hyperbaric oxygen: case report.

A 30-year-old female with a history of seizure disorder and hypoplastic left heart syndrome treated with a Norwood procedure in 1986 followed by a modified non-fenestrated Fontan (Left SVC to IVC to pulmonary arteries) with a known baffle leak presented to the emergency department. On day of presentation, the patient became unresponsive, with perioral cyanosis, rightward gaze and a left facial droop near the end of a platelet transfusion. An emergent non-contrast head CT revealed intracranial air in the right MCA distribution. She was taken to the hyperbaric chamber and was treated with a U.S. Navy Table 6 in a multiplace chamber with no extensions. Ten minutes into the treatment patient became more alert and spontaneously asked questions. The following day she was treated with a U.S. Navy Table 5. Patient had repeat CT of the head, which showed resolution of intracerebral gas and small areas of ischemia in right frontal lobe and right caudate. On hospital day five neurologic exam was normal, with 5/5 strength and no residual deficits. Treating the patient was a concern because patient has a single ventricle, in which the pulmonary artery is connected directly to the vena cava. There is very little data regarding the effects of hyperbaric oxygen (HBO2)therapy on single-ventricle physiology. Only two case reports of three pediatric patients treated with HBO2 for CAGE in a similar setting are known. In these cases the patients had improvements in their symptoms following HBO2. These cases and ours indicate HBO2 is feasible and indicated for CAGE in patients with cyanotic congenital heart disease.

Mortality and neurodevelopmental outcome at 1 year of age comparing hybrid and Norwood procedures.

OBJECTIVES: Neonates with hypoplastic left heart syndrome (HLHS) are at risk of high mortality and neurodevelopmental morbidity. As an alternative to Norwood-type stage I palliation, the hybrid procedure has been developed. It consists of bilateral pulmonary artery banding, catheter-based stenting of the arterial duct and balloon atrioseptostomy and delays open-heart surgery. Thus, it may be associated with a better outcome. The aim of this study was to determine the mortality and neurodevelopmental outcome in patients with HLHS and other univentricular heart (UVH) defects treated with hybrid or Norwood procedures. METHODS: Thirty-one children (18 males) with HLHS and other UVH defects undergoing Norwood or hybrid procedure between 2004 and 2008 were consecutively enrolled. Mortality and neurodevelopmental outcome at 1 year of age were determined. RESULTS: One-year mortality was 36% (31% in the hybrid vs. 39% in the Norwood group, P=0.71). Predictors of mortality were lower birth weight (P=0.02), older age at first procedure (P=0.02) and smaller size of ascending aorta (P=0.05). Overall, median psychomotor development index (PDI) and mental development index (MDI) of the Bayley Scales of Infant Development II were lower than the norm of 100 [PDI 57 (49-99), P<0.001; MDI 91 (65-109), P=0.002]. No effect of surgical treatment on neurodevelopmental outcome was found. Predictors of impaired motor outcome were length of hospital stay (LOHS) (P=0.01), lower body weight at second procedure (P=0.004) and female sex (P=0.01). Predictors of impaired cognitive outcome were longer mechanical ventilation time (P=0.03), intensive care unit stay (P=0.04) and LOHS (P<0.001), respectively. CONCLUSIONS: Mortality at 1 year of age is comparable between patients undergoing hybrid and Norwood procedures. Early neurodevelopmental outcome is significantly impaired in patients with both HLHS and other UVH defects. Multicentre randomized studies are needed to determine the long-term neurodevelopmental outcome of children treated with the hybrid procedure.

Síndrome de hipoplasia do coração esquerdo (SHCE): o que fazer e o que não fazer no pré e no pós-operatório / Hypoplastic left heart heart syndrome (HLHS): what to do and not to do in the pre-and postoperative care

A síndrome de hipoplasia do coração esquerdo (SHCE) é a cardiopatia congênita que sofreu as mais dramáticas mudanças na avaliação diagnóstica, manuseio e evolução na era recente. Embora o tratamento estagiado da SHCE esteja bem fundamentado na atualidade, ainda ocorre uma grande variabilidade no manuseio desta doença e os bons resultados globais são obtidos por um número reduzido de centros mundiais. O recém-nascido com síndrome de hipoplasia do coração esquerdo apresenta alto risco de choque antes e após o procedimento paliativo inicial. A síndrome de baixo débito cardíaco, caracterizada pela baixa liberação sistêmica de oxigênio, é achado típico após cirurgia cardíaca neonatal. O edema miocárdico e a disfunção pós-isquêmica resultam em baixo débito cardíaco. O recém-nascido com SHCE apresenta outros problemas: a massa ventricular total é reduzida, a anatomia em paralelo da circulação sistêmica e pulmonar resulta em saturação baixa de oxigênio no sangue arterial, além da necessidade do ventrículo único direito prover o débito cardíaco sistêmico e pulmonar. Ocorre, também, a necessidade de equilibrar as resistências periférica e pulmonar, com ajuste de drogas e ventilação, balanceando as duas circulações. O manuseio perioperatório destes pacientes com alto risco de mortalidade depende de avaliações constantes do paciente, com diagnóstico e condutas precisas. Este assunto é aqui abordado, baseado em nossa experiência com alto índice de sucesso e em trabalhos publicados por centros mundiais com excelentes resultados no tratamento desta doença desafiadora.