ABSTRACT Clinical data: Infant, nine months of age, female, diagnosed with congenital heart disease, with signs of heart failure associated with cyanosis and difficulty in gaining weight. Chest radiography: Cardiomegaly with prevalence of pulmonary vascular network. Electrocardiogram: Ectopic atrial rhythm with right ventricular overload and left anterosuperior divisional block. Echocardiogram: Single atrium with absent interatrial septum, atrioventricular connection with a single valve and two orifices, with increased pulmonary pressure and high Qp/Qs. Computed tomography: Absence of portal vein and intrahepatic segment of the inferior vena cava. Infrahepatic portion continuing with the azygos system at the level of the thoracic cavity, presence of mesenteric-caval communication associated with signs suggestive of hepatic peribiliary fibrosis. Diagnosis: Abernethy malformation is a rare condition and represents an extrahepatic portosystemic shunt that develops between the mesenteric-portal vasculature and the systemic veins. It may be associated with cardiac malformations and advance with pulmonary hypertension and even the need for liver transplantation. Persistent cyanosis after corrective surgery led to a deeper investigation and correct diagnosis of this malformation. Operation: Sternotomy with 68 minutes of cardiopulmonary bypass and nine minutes of total circulatory arrest. In the postoperative period, persistence of cyanosis was evident, even though there were no immediate complications. Patient was discharged on the 10th postoperative day. An abdominal computed tomography angiography confirmed the diagnosis of Abernethy type I malformation, and the patient was transferred for liver transplantation after congenital heart disease treatment.

ABSTRACT Clinical data: Infant, nine months of age, female, diagnosed with congenital heart disease, with signs of heart failure associated with cyanosis and difficulty in gaining weight. Chest radiography: Cardiomegaly with prevalence of pulmonary vascular network. Electrocardiogram: Ectopic atrial rhythm with right ventricular overload and left anterosuperior divisional block. Echocardiogram: Single atrium with absent interatrial septum, atrioventricular connection with a single valve and two orifices, with increased pulmonary pressure and high Qp/Qs. Computed tomography: Absence of portal vein and intrahepatic segment of the inferior vena cava. Infrahepatic portion continuing with the azygos system at the level of the thoracic cavity, presence of mesenteric-caval communication associated with signs suggestive of hepatic peribiliary fibrosis. Diagnosis: Abernethy malformation is a rare condition and represents an extrahepatic portosystemic shunt that develops between the mesenteric-portal vasculature and the systemic veins. It may be associated with cardiac malformations and advance with pulmonary hypertension and even the need for liver transplantation. Persistent cyanosis after corrective surgery led to a deeper investigation and correct diagnosis of this malformation. Operation: Sternotomy with 68 minutes of cardiopulmonary bypass and nine minutes of total circulatory arrest. In the postoperative period, persistence of cyanosis was evident, even though there were no immediate complications. Patient was discharged on the 10th postoperative day. An abdominal computed tomography angiography confirmed the diagnosis of Abernethy type I malformation, and the patient was transferred for liver transplantation after congenital heart disease treatment.

Resumo Introdução Os Cuidados Paliativos (CP) buscam oferecer uma melhor qualidade de vida para pacientes com doenças em fase avançada. No entanto, há poucos dados sobre sua aplicação e demanda na atenção primária. Assim, buscou-se explorar um método de triagem para identificar pacientes com indicação de CP na Estratégia Saúde da Família (ESF), quantificar o número desses pacientes no território de uma Unidade Básica de Saúde (UBS) e descrever suas características clínicas e sociodemográficas. Métodos Pacientes cadastrados na ESF foram triados por meio da Palliative Care Screnning Tool para identificar aqueles com necessidade de CP, e foi aplicado um questionário para a obtenção das informações. Resultados Após a triagem, 24 pacientes foram incluídos (0,73% da população cadastrada), com idade média de 76 anos. Condições crônicas foram as mais frequentes, principalmente devido às doenças cerebrovasculares, cânceres e demências. A maioria requeria assistência nas atividades cotidianas, e os serviços mais utilizados na UBS foram a obtenção de medicamentos e a orientação técnica. Identificaram-se limitações no suporte oferecido pela UBS. Conclusões Não há iniciativas específicas para a oferta de CP na ESF, apesar de haver pacientes com essas demandas, com alto grau de incapacidade funcional. As políticas para a aplicação de CP são limitadas na atenção primária.

Abstract Background Palliative Care (PC) aims to offer a better quality of life to patients with advanced stage diseases but there is few data about its application and requirements in the primary care. Thus, a screening method was performed to identify patients with need for PC in the Brazilian Family Health Strategy (ESF), to quantify how the number of patients in a Basic Health Unit (UBS) and to describe their clinical and sociodemographic characteristics. Methods ESF patients were screened by Palliative Care Screening Tool to identify the need for PC and a questionnaire was applied to obtain data. Results Screening method resulted in 24 patients included (0.73% of the ESF registered population), with average age of 76 years old. Chronic conditions were most frequent, especially due to stroke, cancer and dementia. Most patients required considerable assistance in daily activities, and the most usual support offered by UBS was drugs and technical orientation. We identified limitations in the received support from UBS. Conclusion There is no specific initiative to offer PC in the ESF despite some patients’ needs, with high level of functional incapacity. The specific polices to support the PC application in primary care are limited.