Anomalous origin of the left coronary artery from the pulmonary artery: echocardiographic diagnosis / Origen anómalo de la arteria coronaria izquierda de la arteria pulmonar: diagnóstico ecocardiográfico

The experience with echocardiographic diagnosis of five cases of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) is reviewed. MATERIAL AND METHODS: all cases with a diagnosis of ALCAPA during a 10 year period were included. RESULTS: two age groups were clearly identified: infants and older patients. In the former, the echocardiographic findings included a dilated left ventricle with low ejection fraction (19% and 26%), mitral regurgitation, dilated right coronary artery and difficulties in identifying the origin of the left coronary artery. An 11-year-old asymptomatic boy, a 17-year-old young woman with dyspnea on effort and a 55-year-old woman with angina formed the older group. In these three cases, an abnormal upward flow was detected within the ventricular septum related to the collaterals and the inverse flow of the anterior descending artery. A reverse flow within the origin of the left coronary artery, probably related to an origin in the pulmonary artery, was observed. In all five cases the diagnosis was corroborated using selective right coronary artery angiography. CONCLUSIONS: In the infants, the dilated left ventricle with impaired systolic function, mitral regurgitation and dilated right coronary artery make it necessary to discard the ALCAPA diagnosis. In the older group, even in asymptomatic patients, an abnormal ascending flow within the ventricular septum, combined with a dilated coronary artery, was the most reliable echocardiographic evidence for a diagnosis of ALCAPA.

Fistula coronaria congénita: presentación de siete casos y revisión de la literatura / Congenital coronary fistula: clinical presentation in 7 cases and literature review

Congenital coronary artery fistulas are uncommon anomalies, however themselves may resemble the whole spectrum of cardiac manifestations. Clinical presentations vary considerably from adults to children. MATERIAL AND METHODS: patients with coronary artery fistula diagnosed by selective coronariography in the period from 2000 to 2007 were included. RESULTS: 7 cases were found, 4 children, mean age 6.5 years, and 3 adults, mean age 25.6 years. The fistulae originate from the left coronary artery in 3 children, 2 connect to the pulmonary artery an one to the right atrium, in one child the fistula originated from the right coronary artery and terminated at the right ventricle. In the 3 adult patients, the fistula originated from the right ventricle, two connected to the right ventricle and one to the coronary sinus. Two children were operated on successfully and are asymptomatic 1 and 4 years later. In an adult patient with surgical ligation was performed, 4 weeks later developed an uncomplicated myocardial infarction. We reviewed the literature available and were compared with our cases. CONCLUSIONS: The clinical manifestations and hemodynamic features differ in childhood and adulthood. Coronary angiography is the primary diagnostic tool. Surgical treatment has low mortality and morbidity.

El método de Fick subestima el cálculo deflujo pulmonar en los enfermos operados de Glenn bidireccional / The Fick method underestimates pulmonary flow calculation in patients subjected to bidirectional Glenn procedure

In patients with Bidirectional Glenn who have undergone surgery, the superior caval venous flow provides the only pulmonary blood supply. This is the effective pulmonary flow and at the same time its volume is not enough to overflow the single ventricle. The unsaturated, inferior vena cava flow is not oxygenated, since it goes across the interatrial septal communication and gets mixed in the left ventricle with the pulmonary venous blood. In this work, a bidirectional Glenn case is analyzed. The hemodynamic data before and after the operation are shown. It was evident from this case that the use of the Fick method to measure pulmonary flow in patients with bidirectional Glenn operation is not appropriate. Alternative methods, such as Doppler echocardiography and Magnetic Resonance Imaging, are recommended. A literature review on this subject was carefully done.

Rabdomioma cardiaco tratado quirúrgicamente con éxito y revisión de la literatura / Cardiac rhabdomyoma surgically treated with success. Review of literature

Los tumores cardiacos primarios son raros, con incidencia variable en todas las edades del 0.005 al 0.05%. En pacientes pediátricos, la incidencia es del 0.27%. Los tumores más frecuentes durante la infancia son los rabdomiomas cardiacos, considerados como benignos. Aunque la expresión clínica es amplia, en la mayoría de los casos son asintomáticos y se detectan por la presencia de soplos. En la etapa prenatal se manifiestan con arritmias o hydrops fetalis. En algunos neonatos y lactantes se encuentran arritmias, datos de bajo gasto cardíaco o muerte súbita. La asociación con esclerosis tuberosa se ha observado hasta en un 81%. Se presenta el caso de paciente masculino neonato, con diagnóstico de rabdomioma cardiaco que inicialmente estaba asintomático, sin embargo en el seguimiento requirió de tratamiento quirúrgico a los 5 meses de edad, por datos de insuficiencia cardiaca secundaria a obstrucción del tracto de salida del ventrículo derecho. A 5 meses de la cirugía, el paciente está asintomático.

The primary cardiac tumors are inusual, the incidence varies in all the ages between 0.005 to 0.05%. In pediatrics patients the incidence is 0.27%. The more frequent tumors during the childhood are the cardiac rhabdomyomas. These tumors are considered benigns. The clinical expression is wide, in the most the cases, the patients are asymptomatic and are detected by murmurs. In the prenatal age are manifested by arrhythmias or hydrops fetalis. The neonates and children may be show cardiac arrhythmias, low cardiac index and sudden cardiac death. The association with tuberous sclerosis had been reported in 81%.We presenta neonate with cardiac rhabdomyoma diagnosed in the newborn period when he was asymptomatic, however in the follow-up he developed cardiac failure by obstruction in the out flow tract of the right ventricle. He underwent open cardiac surgery to resect the obstruction. Five months after surgery, the patient remain asymptomatic.

Valvuloplastía pulmonar con balón, experiencia de 15 años en el Centro Médico Nacional Siglo XXI IMSS / Balloon pulmonary valvuloplasty: 15-years experience at the Centro Médico Nacional "Siglo XXI", IMSS

Objetivo: Evaluar la experiencia de 15 años, de la valvuloplastía pulmonar con balón en el Centro Médico Nacional Siglo XXI. Material y métodos: Se estudiaron 150 pacientes 73 (48.7%) masculino y 77 (51.7%) femenino. La edad promedio de los 141 en quienes se realizó la valvuloplastía pulmonar con balón fue de 10.5 ± 11.3 años. Resultados: El gradiente promedio prevalvuloplastía de 86 ± 35 descendió a 21.67 ± 12.20 mm Hg p < .001. La presión sistólica del ventrículo derecho de 106 ± 34.8 mm Hg disminuyó a 53 ± 27 mm Hg p < 0.0001. El gradiente valvular pulmonar en el seguimiento promedio de 48 ± 44 meses fue de 13.43 ± 8.73 mm Hg p < 0.001. Se presentaron complicaciones en 9 (6%) pacientes. Se obtuvo éxito técnico inmediato post en 111 (74%) y fracaso inmediato en 39 (26%). Al final del seguimiento se obtuvo éxito en 104 (89.6%) y fracaso en 12 (10.4%) pacientes, p < 0.0001, con una mortalidad global de 2 (1.33 %) pacientes. Las variables significativas para fracaso fueron: la edad de, 1.5 ± 1.33 años para falla de procedimiento, p < .004; morfología valvular displásica p < 0.001. gradiente valvular pulmonar prevalvuloplastía elevado con p < 0.002 y presión sistólica del ventrículo derecho pre valvuloplastía elevados. p < 0.0001, no descendiendo ambos en el post inmediato. Conclusión: La valvuloplastía pulmonar con balón es el tratamiento de elección, efectivo y seguro de la estenosis valvular pulmonar congénita.

Objective: To evaluate 15 years of experience with balloon pulmonary valvuloplasty in a single third level health care center. Material and methods: Hundred-fifty patients underwent the procedure, 73 (48%) men and 77 (52%) women, mean age 10.5 ± 11.3 years. Results: The initial systolic gradient decreased from 86 ± 35 to 21.67 ± 12.20 mm Hg, p < 0.001, whereas the initial right ventricular systolic pressure decreased from 106 ± 34-8 to 53 ± 27 mm Hg, p < 0.0001. At the end of the follow-up, 48 ± 44 months, the systolic gradient was 13.43 ± 8.73 mm Hg, p < 0.001. Major complications occurred in 9 (6.4%) patients. Immediate technical success was achieved in 111 (74%) patients and failure in 39 (26%). At the end of the follow-up period, successful outcomes were achieved in 104 (89.6%); in contrast, failures were present in 12 (10.4%) patients, p < 0.001. Death occurred in 2 (1.33%) patients. The predictors for failure were age < 1.5 ± 1-33 years (p < 0.004), dysplastic valve (p < 0.001), high initial systolic gradient (p < 0.002), and high initial systolic right ventricular pressure (p < 0.0001). Conclusion: Balloon pulmonary valvuloplasty is an effective, safe, and first choice treatment for congenital pulmonary valve stenosis. (Arch Cardiol Mex 2003; 73:190-196).