Hipertensão arterial pulmonar associada às cardiopatias congênitas / Pulmonary arterial hypertension associated to congenic heart disease

As cardiopatias congênitas estão alocadas dentro do grupo 1 da classificação da hipertensão pulmonar (HP), junto com a hipertensão arterial pulmonar (HAP) idiopática, mas as doenças que causam hipertensão pulmonar devido à doença do corações esquerdo, incluindo algumas cardiopatias congênitas, estão nomeadas no grupo 2. A síndrome de Eisenmenger caracteriza-se pelo aumento da pressão pulmonar a níveis sistêmicos e shunt da direita para a esquerda ou bidirecional devido a um grande defeito, tendo como marca a intensa cianose. Estes pacientes apresentam progressão lenta dos sintomas e alteração de coagulação podendo evoluir tanto com trombose como com sangramento. Devem ser orientados a se manterem bem hidratados e ativos, de forma a reduzir a hipercoagulabilidade sanguínea. Os pacientes devem ser tratados com reposição de ferro quando necessário e flebotomias para redução do hematócrito devem ser evitadas. Vasodilatadores pulmonares como a bosentana, a sildenafila e o iloprost demonstraram ser capazes de reduzir a resistência vascular pulmonar e melhorar a classe funcional em pequenos grupos de pacientes com cardiopatias congênitas, podendo ser usados com segurança em pacientes com síndrome de Eisenmenger. Por outro lado, não há estudos controlados quanto ao uso de vasodilatadores pulmonares em pacientes com shunt esquerdo-direito...

Congenital heart defects are allocated within the Group 1 of pulmonary hypertension (PH) classification along with pulmonary arterial hypertension (PAH) Idiopathic, but the diseases that cause pulmonary hypertension due to left heart disease, including some congenital heart diseases, are named in group 2. The Eisenmenger syndrome is characterized by increased pulmonary and systemic pressure levels with right to left or bidirectional shunt due to a large defect, producing intense cyanosis. These patients have slow progression of symptoms and changes of coagulation may progress with both thrombosis and bleeding. Patients should be advised to stay well hydrated and active in order to reduce blood hypercoagulability. Patients should be treated with iron replacement when necessary. Phlebotomy to reduce the hematocrit should be avoided. Pulmonary vasodilators such as bosentan, sildenafil and iloprost proved capable of reducing pulmonary vascular resistance and improved functional class in small groups of patients with congenital heart disease and can be used safely in patients with Eisenmenger syndrome. On the other hand, there is no controlled studies to suport the use of pulmonary vasodilator in patients with left-right shunt...

Cor triatriatum sinister e hipertensão arterial pulmonar secundária em cão / Cor triatriatum sinister and secondary pulmonary arterial hypertension in a dog

This paper describes the occurrence of cor triatriatum sinister, a rare cardiac malformation in dogs, associated with pulmonary edema and pulmonary hypertension in a 5-year-old Poodle female with history of acute dyspnea and cyanosis. The animal presented acute respiratory failure, heart failure with low cardiac output, progressing to acute tubular necrosis and death. The diagnosis was made posmortem due to the clinical instability of the dog. This malformation was diagnosed by the subdivision of the left atrium into two compartments separated by an abnormal fibromuscular membrane, absence of structural abnormalities of the mitral valve and thickening of pulmonary artery tunica media associated with renal tubular degeneration. The occurrence of cor triatriatum in dogs is most common in the right atrium, defined as cor triatriatum dexter. Additionally, pulmonary arterial hypertension associated with this malformation is described only in humans with this heart defect.