Graça Sousa

Coronary and systemic aneurysms in an infant with Kawasaki disease.

Kawasaki disease is an immune-mediated vasculitis of unknown etiology. Among untreated patients, the incidence of coronary aneurysm reaches 25%, declining to 5% even among those treated appropriately [1]. The incidence of iliac artery aneurysms varies from 0.6% to 2.5% [2]. The aneurysms tend to regress with medical therapy but are more prone than other systemic aneurysms to the development of stenotic lesions [3]. In this case, Dacron graft replacement is required [2]. We describe the case of a previously well 6-month-old infant admitted to our intensive care unit with cardiac failure after a 10-day history of unremitting fever of unknown origin and a fading macular papular rash. No other clinical signs were apparent. An echocardiogram showed a moderate pericardial effusion, diminished left ventricular contractility, and moderate to large aneurysms in both coronary arteries. The largest aneurysm (9 mm) was in the right coronary artery. Immunoglobulin (2 g/kg) and acetyl salicylic acid (100 mg/kg/day) were started, and 4 days after admission the infant required resuscitation for cardiac arrest. At the time, the electrocardiogram showed evidence of posteroinferior ischemia, and a repeat echocardiogram showed the presence of thrombi in the coronary aneurysms. He was managed with thrombolytic therapy (alteplase 0.3 mg/kg/h for 6 h) and repeat immunoglobulin (2 g/kg), with reestablishment of flow in the obstructed aneurismal coronary arteries and subsequent clinical improvement. The infant was clinically well 1 month after discharge, with no further cardiac events reported. However, followup echocardiography showed no regression of the coronary aneurysms. Cardiac catheterization performed to define the coronary lesions showed rosary-like dilations of the right (Fig. 1) and left coronary arteries as well as sac-like aneurysms of both internal iliac arteries (Fig. 2). No other cardiac anomalies were found, and left ventricular function was preserved.

Right Subclavian Artery Aneurysm in an Adolescent With a Bicuspid Aortic Valve

Bicuspic aortic valve is the most common congenital cardiac anomaly (Fedak et al. 106:900–904, 2002), and it is associated with other structural anomalies of the aorta, such as coarctation, suggesting a common embryologic developmental mechanism (Mergan et al. 104:118–119, 2004). In more than a half of patients, it is associated with progressive dilation and aneurysm formation of the aorta (Warnes 89:965–966, 2003) despite normally functioning bicuspid aortic valves. In this context, aneurysms of the right subclavian artery are extremely rare, and even more so when associated with a right-sided aortic arch that has a left aberrant subclavian artery with a Kommerell diverticulum, as found in the reported case. These aneurysms represent a significant risk for thromboembolism and rupture, and elective surgical management should be advised, even for asymptomatic cases.

Major aortopulmonary collateral artery (MAPCA) gigante – causa rara de sopro em recém‐nascidos

As major aortopulmonary collateral arteries (MAPCA) são vasos anómalos que saem da aorta ou dos seus ramos principais e que irrigam com sangue oxigenado o parênquima pulmonar. Habitualmente encontram-se associados a cardiopatia congénita, sendo ainda mais rara a sua ocorrência isolada. Descreve-se o caso clínico de um recém-nascido do sexo feminino, de termo, sem antecedentes de relevo em que é detetado sopro III/VI no bordo esternal esquerdo no primeiro dia de vida. Aos sete dias de vida é observada em teleconsulta de cardiologia onde é suspeitada estenose da veia pulmonar inferior direita, apesar de PSAP normal. A ecografia cardíaca no HPCM mostrava «vaso anómalo a drenar na veia pulmonar inferior direita». Com um mês realiza AngioTAC, que revelou MAPCA única a emergir do tronco celíaco para o pulmão direito, drenando na veia pulmonar inferior direita (Figura 1).

Endocardite infecciosa como primeira manifestação de cardiopatia congénita de apresentação tardia

A diagnosis of congenital heart disease is usually established at an early age, so infective endocarditis is a rare form of presentation. The authors describe the case of a male adolescent with a week-long history of intermittent fever and unquantified weight loss. Physical examination detected pansystolic and diastolic murmurs, and an associated precordial thrill. Laboratory tests showed evidence of an active infection. Etiological investigation revealed a perimembranous ventricular septal defect, aortic regurgitation, and aortic and mitral valve vegetations. A diagnosis of mitral-aortic infective endocarditis was made and he was started on intravenous antibiotics and anticongestive therapy. After initial clinical improvement, he developed symptoms and signs of congestive heart failure. Repeat echocardiography showed an extensive mitral-aortic paravalvular abscess. The antibiotics were changed and anticongestive therapy was intensified, and he subsequently underwent surgery. The outcome has been generally favorable, and at present he is asymptomatic under anticongestive therapy.

Tumor cardíaco em criança assintomática: um diagnóstico acidental

Cardiac tumors in the pediatric population are rare, their incidence range between 0.001% and 0.003%. They are mostly benign, rhabdomyomas the most common type, followed by fibromas. The clinical features are being usually nonspecific and depend on the size and location of the tumor within the heart. We report the case of a previously healthy four-year-old boy referred for flu-like symptoms. A respiratory infection was suspected and a chest X-ray showed an increased cardiothoracic index. An echocardiogram revealed a single large heterogeneous mass in the left ventricle emerging from the lateral wall. Despite its size, the mass did not obstruct the left ventricular outflow tract or affect mitral valve function. Cardiac magnetic resonance imaging showed a large mass whose imaging features were suggestive of a fibroma. He became symptomatic during follow-up and was referred for surgical excision of the mass. Histological study confirmed a fibroma. At present the patient remains asymptomatic.Resumo Os tumores cardiacos sao uma entidade rara em idades pediatricas, com uma incidencia variavel entre 0,001% e 0,003%. Sendo habitualmente benignos, os rabdomiomas e os fibromas sao os tipos histologicos mais frequentes. A clinica e inespecifica e dependente das dimensoes e localizacao do tumor. Descreve-se o caso de uma crianca de quatro anos previamente saudavel e assintomatica, observada em contexto de infeccao respiratoria, tendo-se identificado cardiomegalia na teleradiografia toracica. A ecocardiografia transtoracica revelou uma massa volumosa e heterogenea no ventriculo esquerdo, sem causar obstrucao da câmara de saida ou disfuncao valvular. A ressonância magnetica foi sugestiva de fibroma. Procedeu-se a terapeutica cirurgica, com excisao completa que confirmou fibroma mantendo-se assintomatica.

[Asymptomatic cardiac tumor in a child: an incidental diagnosis].

Abstract Cardiac tumors in the pediatric population are rare, their incidence range between 0.001% and 0.003%. They are mostly benign, rhabdomyomas the most common type, followed by fibromas. The clinical features are being usually nonspecific and depend on the size and location of the tumor within the heart. We report the case of a previously healthy four-year-old boy referred for flu-like symptoms. A respiratory infection was suspected and a chest X-ray showed an increased cardiothoracic index. An echocardiogram revealed a single large heterogeneous mass in the left ventricle emerging from the lateral wall. Despite its size, the mass did not obstruct the left ventricular outflow tract or affect mitral valve function. Cardiac magnetic resonance imaging showed a large mass whose imaging features were suggestive of a fibroma. He became symptomatic during follow-up and was referred for surgical excision of the mass. Histological study confirmed a fibroma. At present the patient remains asymptomatic.