Montserrat Bret Zurita

Evaluación de las cardiopatías congénitas en el adulto

Los avances en el diagnostico y tratamiento quirurgico de las cardiopatias congenitas (CC) durante la edad pediatrica estan provocando un incremento excepcional de su prevalencia durante la vida adulta. Las CC en el adulto representan un desafio diagnostico nuevo para el cardiologo clinico, poco familiarizado con la complejidad anatomica y funcional de las malformaciones cardiacas. La evaluacion con tecnicas de imagen de las CC del adulto puede llegar a ser tan precisa como en el nino, pero estas tecnicas no pueden sustituir a una valoracion clinica detallada. La exploracion fisica, el ECG y la radiografia de torax siguen siendo los tres pilares basicos en los que se sustenta el diagnostico clinico. La ecocardiografia transtoracica es, sin duda, la tecnica de imagen que aporta mayor informacion y, en muchos casos, suficiente. Pero los pacientes adultos no tienen tan buena ventana ultrasonica como los ninos, y las intervenciones quirurgicas previas anaden mayor deterioro de la imagen. Aunque las nuevas tecnologias ultrasonicas, como la imagen armonica o la ecocardiografia de contraste, tratan de solventar esta diferencia, con frecuencia es necesario recurrir a otros metodos diagnosticos. Afortunadamente, la ecocardiografia transesofagica y la resonancia magnetica son mas faciles de realizar de forma rutinaria en el adulto que en el nino, por cuanto no precisan anestesia. Estas tecnicas, junto con la cardiologia nuclear y el cateterismo cardiaco, completan los metodos diagnosticos de segundo nivel disponibles actualmente. Para evitar exploraciones innecesarias, el cardiologo clinico debe elegir bien la secuencia de tecnicas diagnosticas, cuya informacion en muchos casos es redundante, pero en otros complementaria. Este articulo trata de comparar el valor diagnostico de las diferentes tecnicas de imagen en el adulto con CC con o sin intervencion quirurgica previa.

Multiple congenital ectatic and fusiform arterial aneurysms associated with lower limb hypoplasia

Aneurysmal disease is uncommon in children, and its presence often leads to suspicion of genetic disorders (Loeys-Dietz syndrome, Marfan syndrome, Ehlers-Danlos syndrome, tuberous sclerosis), trauma, and infection. We describe the case of a newborn boy with generalized left lower limb hypoplasia associated with diffuse areas of arteriectasis combined with areas of stenosis and fusiform aneurysms of the iliac, femoral, and popliteal arteries. No additional vascular territories were affected. The patient was asymptomatic, and no therapeutic intervention has been considered. Numerous complementary imaging and laboratory examinations failed to establish a definitive diagnosis. This collection of findings has not been previously reported.

Images in cardiologyLarge Congenital Coronary FistulaFístula coronaria congénita gigante

A newborn infant, born following an unmonitored pregnancy to a cocaine-using mother, was referred to our cardiology service due to an episode of peri-oral cyanosis with apnea lasting a few seconds and spontaneous recovery. Heart and lung auscultation was unremarkable and there was no hepatomegaly. An echocardiogram showed elevated pulmonary pressures attributable to transitional neonatal circulation, permeable foramen ovale and systolic-diastolic flow that crossed the left atrioventricular groove and appeared to terminate in the right ventricle (RV). Coronary arteries had a normal pattern, with a normal right coronary artery in the initial portion and mild dilatation of the left coronary artery (LCA). There was no chamber dilatation or segmental defects of contractility. The electrocardiogram showed no evidence of coronary ischemia or significant abnormalities for the patient’s age. Troponin and creatine kinase values were within the normal range, and other parameters were unremarkable. After establishing a diagnosis of suspected coronary fistula, we performed a coronary computed tomography (Figures 1-3) when the patient was aged 23 days. The image was compatible with an arterial fistula to the RV passing through the posterior basal wall of the RV before the origin of the posterior descending coronary artery. The fistula appeared to communicate with the lateral wall of the right atrium (RA), coinciding with an arterial loop in the circumflex artery (CxA). At age 2.5 years, the patient remains cardiologically asymptomatic. He has been treated conservatively and has been monitored through regular outpatient appointments. His weight gain is appropriate and he shows no signs of heart failure. Rev Esp Cardiol. 2018;71(4):292

A coarctation on the wrong aortic arch side.

A 3-month-old male infant, with diagnosis of a complex, cyanotic, congenital heart disease (double-outlet right ventricle, pulmonary stenosis, and hypoplastic pulmonary arteries, among other defects) and a double aortic arch (Fig. 1, [L, left; LCA, left carotid artery; LSA, left subclavian artery; R, right; RCA, right carotid artery; RSA, right subclavian artery]), had required a shunt from ascending aorta to main pulmonary trunk (Fig. 2A, *), plus a section of the aortic arch distal to right subclavian artery (Fig. 2B, y). During admission, the infant experienced low systemic cardiac output, with arterial pressure gradient between upper and lower extremities, and an echo suspicion of aortic coarctation. A thorax computed tomography scan showed a stenotic area in the native, left aortic arch (Figs. 2A, 2B, arrow), bizarrely located between the left carotid artery and the left subclavian artery. Due to the atypical location of the coarctation, and the complexity and risk of the patient, after agreement with cardiac surgeons we accordingly performed a balloon catheter angioplasty to resolve the coarctation (Fig. 3A), which was effective and uneventful (Fig. 3B). Angio computed tomography scan and 3D reconstructions provide fast, high-resolution images of congenital heart disease, with minimum radiation doses and use of intravenous contrasts. In infants with congenital heart disease, computed tomography scan is better than magnetic resonance imaging, especially in large-vessel anomalies. Findings may be later confirmed in the operation theater or the cath-lab. In our patient with double aortic arch intervention, computed tomography scan provided accurate images of an atypical coarctation, and allowed an individualized approach. Figure 1.