Anomalous origin of the left coronary artery from the pulmonary artery. Echocardiographic diagnosis.

UNLABELLED: 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.

[Congenital coronary fistula. Clinical presentation in 7 cases and literature review]. / Fistula coronaria congénita. Presentación de siete casos y revisión de la literatura.

UNLABELLED: 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.

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

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.

[MURCS association: case report]. / Asociación MURCS: reporte de un caso.

Clinical term of association refers to a not randomized congenital malformations which are present in one single subject. The term MURCS is an acronym for (MU) Mullerian, (R) Renal, (C) Cervicothoracic, (S) Somite abnormalities. We communicate a case of a phenotipically normal 16 years old female patient with primary amenorrhea due to müllerian malformations and cervicothoracic dysplasia integrating the MURCS association diagnosis.

[Sequential segmental analysis in 100 cardiopulmonary specimens]. / Aplicación del análisis secuencial segmentario en 100 especímenes cardiopulmonares.

OBJECTIVE: To evaluate the feasibility and accuracy of the sequential segmental analysis (SSA) to diagnose congenital heart diseases. MATERIAL AND METHODS: 100 cardiopulmonar specimens were analyzed according with the sequential segmental analysis. The atrial situs, atrioventricular (AVC) and ventriculoarterial connections (VAC) were defined, finally associated lesions were observed. RESULTS: From 100 specimens, 91 had situs solitus (SS), 6 inversus (SI) and 3 isomerism (2 right isomerism and 1 left isomerism). The AVC in these hearts were: 89 biventricular, 80 (90%) concordant, 6 (6.7%) discordant and 3 (3.3%) ambiguous (2 right topology an 1 left topology). The univentricular AVC were 11, 4 (36%) had absent left AVC (mitral atresia), 3 (27%) absent right AVC (tricuspid atresia), 2 (18%) double inlet right ventricle (DIRV), 1 double inlet left ventricle (DILV) and 1 (9%) double inlet undetermined ventricle. The VAC in SS were: 52 (57%) concordant, 13 (14%) discordant, 6 (6.6%) double outlet right ventricle (DORV), 20 (22%) with single outlet (13 pulmonary atresia , 5 aortic atresia and 2 common arterial trunk). The VAC in SI were: concordant in one, (16.7%), discordant 3 (50%) and DORV in 2 (33.3%). In the hearts with isomerism, the VAC were concordant in one, DORV in one and single outlet in another (pulmonary atresia). The associated anomalies were ASD (67%), PAD (51%) and VSD (53%). CONCLUSIONS: The SSA was and effective and accurate tool to codify clear and completely the congenital heart disease. It is suggested that if the SSA is normal to describe this situation as a Normal Basic Heart Organization and then to describe the associated anomalies.

[Congenital aortic valve stenosis. Current treatment]. / Estenosis valvular aórtica congénita. Actualización del tratamiento.

OBJECTIVE: Congenital aortic valve stenosis is a common lesion, with an approximate incidence of 5 to 7% of all cardiac malformations and occupies the first place among left heart obstructions. In recent years, many modalities of treatment have been developed. Fetal interventions has evolved in one extreme of life, on the other hand, percutaneous aortic valve replacement is now available for aged adults. In children and adolescents, percutaneous aortic valve valvuloplasty is now more effective with new techniques. The Ross procedure is the first choice treatment in children and young adults with hipoplastic aortic annulus. Considerable medical information has evolved and expanded from these techniques. A review of the indications, optimal timing, and outcomes of these procedures is pertinent.

[Pentalogy of Cantrell. A case report]. / Pentalogía de Cantrell. A propósito de un caso.

Ectopia cordis is an extremely rare cardiac anomaly. The heart is localized partially or totally outside the thorax cavity. This anomaly occurs as an isolated defect or combined with others midline defects. Cantrell and colleagues described, in 1958, a syndrome including defects of the abdominal wall, sternum, diaphragm, pericardium and heart. There are few successful surgical cases with this pentalogy. We describe a case with this Cantrell's pentalogy. The cardiac malformation was a univentricular heart with pulmonary stenosis. The patient underwent successful surgical palliation with a systemic-to-pulmonary anastomosis and uneventful recovering.

[Usefulness of office hysteroscopy]. / Utilidad de la histeroscopia de consultorio.

OBJECTIVE: To demonstrate the efficacy and feasibility of the office hysteroscopy in the treatment of the endouterine pathologies. DESIGN: retrospective, descriptive and observational. PATIENTS AND METHOD: We included all the office hysteroscopies performed since February-2000 to August-2004 (n=84) in a private medical office. We performed 32 diagnostic procedures without anesthesia and 52 operative procedures with anesthesia (I.V. sedation). We used a Karl Storz diagnostic telescope and a Bettocchi operative telescope. RESULTS: Postoperative care was in the office since 90 minutes and all patients went home without complications, Mean age of the patients was 38+/-10 years. The indications to perform the procedure were infertility and abnormal transvaginal bleeding. There were not complications. All of the diagnostic procedures were done without anesthesia and we verified patient satisfaction with a pain store since 1 to 10 points. Twenty patients could get pregnant after the procedure and in two patients we detected endometrial carcinoma. CONCLUSION: With these results, we can assume that office hysteroscopy is a procedure with minimal complications when is performed by expert hands and with excellent patients' satisfaction and benefit.

[Balloon stabilization by means of cardiac stimulation during aortic valvuloplasty]. / Estabilización del balón mediante estimulación cardíaca en la valvuloplastía aórtica.

OBJECTIVE: To evaluate rapid ventricular pacing in balloon aortic valvuloplasty, an initial strategy to achieve balloon stability. MATERIAL AND METHODS: From September to December 2004, a prospective protocol was started: three male consecutive patients with aortic valve stenosis were treated by this strategy. Age of the patients were 13, 6 and 5 years old. All had a bipoplar pacing catheter placed in the right ventricle. Invasive systemic pressures were documented with a catheter in the descending aorta. Rapid ventricular pacing was initiated at the rate of 150 per minute and increased to a rate required to achieve a drop in systemic pressure by 50%. The balloon was inflated only after the pacing rate was reached and the blood pressure dropped. Pacing was continued until the balloon was completely deflated. RESULTS: The systolic gradients across the aortic valve before balloon dilatation were 90, 110 and 55 mmHg. The systolic pressures in aorta were 90 and 110 mmHg. The pacing rate to drop the pressure by 50% were 170, 250 and 220 per minute. The pacing time was 15 seconds in all patients. Balloon stability at time of inflation was achieved in all cases with no balloon movement. The post-ballooning gradients were 23, 28 and 15 mmHg. Angiogram performed post balloon dilatation showed no change compared with the pre-balloning angiogram in aorta: trivial aortic incompetence in the first case and none in the second and third cases. CONCLUSIONS: Rapid ventricular pacing to stabilise the balloon during balloon aortic valvuloplasty seems to be safe and effective and may decrease the incidence of aortic incompetence.

Types of obstructions in double-chambered right ventricle: mid-term results.

BACKGROUND: The double-chambered right ventricle (DCRV) is increasingly recognized as a distinct obstruction entity. The nature of the obstruction is not well defined. METHODS: Patients with DCRV were prospectively studied during the last 4 years according to the following criteria: 1) pressure gradient by echo Doppler and cardiac catheterization within the right ventricle; 2) angiographic demonstration, and 3) surgical confirmation. RESULTS: From March 1997 to March 2001, 10 new cases were included. Age ranged from 2 to 14 years (mean 9.5 +/- 4.4 years), weight ranged from 9.9 to 75 kg (mean 23 +/- 13.6 kg), and height from 0.85 to 1.48 m (mean 114 +/- 19 cm). Systolic gradient by echo Doppler ranged from 20 to 135 mmHg (mean 86 +/- 44 mmHg) and by cardiac catheterization, 18 to 130 mmHg (mean 78 +/- 35 mmHg). In terms of angiographic findings, in six patients the right ventriculogram showed an oblique and low obstruction; in four patients the obstruction was high and horizontal. With regard to surgical findings, angiographic findings were confirmed by the surgeon except in one patient, in whom both types of obstruction were present. No mortality was observed. With follow-up 4 to 40 months after surgery (mean 24 +/- 15 months), 8 of 10 patients were evaluated; all corresponded to class I NYHA. Systolic gradient by echo Doppler ranged from 0 to 11 mmHg (mean 4 +/- 6 mmHg). CONCLUSIONS: DCRV IS PRODUCED BY THE FOLLOWING THREE TYPES OF MUSCULAR OBSTRUCTIONS: low and oblique obstruction; high and horizontal obstruction, and mixed obstruction. Mid-term surgical results are satisfactory.

Intraluminal aortoplasty vs. surgical aortic resection in congenital aortic coarctation. A clinical random study in pediatric patients.

BACKGROUND: Our objective was to compare results of two therapeutic modalities to treat congenital aortic coarctation: intraluminal aortoplasty without endoluminal stent installation (patients in group A) vs. surgical aortic resection (patients in group B). Trans-coarctation gradient pressure was evaluated prior to and immediately after treatment. Re-coarctation, aneurysm formation, in-hospital morbidity and mortality, and complications related to treatment were also evaluated. METHODS: A clinical, randomized, multicenter study was performed in pediatric patients with congenital aortic coarctation. Immediate and mid- to late therapeutic results were evaluated. With regard to statistics, we evaluated event variations by Kaplan-Meier model, nonparametric Wilcoxon test, Mann-Whitney U test, two-tailed Student t and chi-square tests, and Fisher analysis. Significance was considered relevant when p<0.05. RESULTS: There were no differences in demographic variables, procedure failure, complications, mortality, or aortic aneurysm between groups A and B, respectively. Intraluminal angioplasty and surgical aortic resection were similarly effective in reducing trans-coarctation pressure gradient, as well as arterial systemic pressure. However, differences were found between groups A and B at follow-up. Group A showed higher re-coarctation (50 vs. 21%). Absence of peripheral arterial pulses in limbs was higher in group A (50 vs. 21%), as well as persistence of arterial hypertension (49 vs. 19%); these differences were significant (p<0.05). On the other hand, complications observed after surgical aortic resection were more serious than post-angioplasty complications, but these differences were not statistically significant. CONCLUSIONS: Although re-coarctation and persistency of arterial hypertension were less frequent after surgical aortic resection, complications observed with this procedure are more serious than complications related to angioplasty, although these differences are not statistically significant.

[Stenting and redilatation of the ductus arteriosus in univentricular heart with pulmonary atresia. A case report]. / Stent y redilatación con globo del conducto arterioso en ventrículo único con atresia pulmonar. Descripción de un caso.

Complex congenital heart disease with pulmonary atresia is ductus arteriosus-dependent. Two palliative treatments have been available for years: a systemic pulmonary shunt and prostaglandin E1 infusion. Recently, interventional cardiology has offered a new procedure for these patients: stenting of the patent arterial duct. We report a case with univentricular heart and pulmonary atresia in which percutaneous balloon angioplasty and stenting of the arterial ductus were performed successfully. Nineteen months later the patient is in good clinical conditions with 75% of oxygen saturation.

Transcatheter closure of secundum atrial septal defects and fenestrated Fontan using the Amplatzer septal occluder. Initial prospective study.

OBJECTIVE: To evaluate the safety and efficacy of transcatheter closure of secundum atrial septal defects and fenestrated Fontan with the Amplatzer septal occluder. METHODS: Fifteen consecutive patients, with a significant interatrial communications, were considered for the procedure; four patients with defects that were too large or with deficient margins were excluded after initial transesophageal echocardiography. RESULTS: Eleven procedures were performed in 11 patients (10 atrial septal defects and 1 fenestrated Fontan) aged 9 to 38 years, mean 17.7 +/- 9 years; body weight 30 to 87 kg, mean 51.4 +/- 16. The stretched balloon diameter of the defects ranged from 8 to 28 mm, mean 18.8 +/- 6.9; the diameter of the devices ranged from 10 to 30 mm, mean 20.8 +/- 6. Immediate total occlusion rate was 18.1%, rising to 63.6% after 24 hours. Total occlusion rate at one month reached 100%. Severe transient sinus bradycardia in one (9%) was the only complications. At follow-up (10 to 26 months, mean 13.2 +/- 5.0) all patients remain asymptomatic with no residual shunt. CONCLUSIONS: The Amplatzer septal occluder is very efficient and offered interventional interatrial communications closure in 100% of our group of consecutive patients with excellent intermediate results.

[Scimitar syndrome associated to pulmonary atresia with interventricular communication. First reported case]. / Síndrome de cimitarra asociado a atresia pulmonar con comunicación interventricular. Primer caso reportado.

We present the case of a 15 months-old male with Scimitar Syndrome associated with ventricular septal defect and pulmonary atresia. The diagnosis was made by cardiac catheterization and angiography and was confirmed by the necropsy. Clinical and surgical considerations of this exceptional association were made. To the best of our knowledge this is the first case reported in the relevant literature.

Tricuspid atresia associated with common arterial trunk and 22q11 chromosome deletion.

The case of a four-months old male with coexistent tricuspid atresia and common arterial trunk is presented. The diagnosis was made by cardiac catheterization and selective angiocardiography. Clinical considerations are discussed and the review of the available literature reveals this patient to be the tenth case reported of this very unusual association of cardiovascular defects, and the first with positive deletion of the 22q11 chromosome.

[Congenital percutaneous aortic valvuloplasty in 70 subjects with congenital aortic stenosis, mid-term results]. / Valvuloplastia aórtica percutánea en 70 enfermos con estenosis valvular aórtica congénita. Resultados a mediano plazo.

OBJECTIVE: To evaluate mid-term results with Percutaneous aortic valvuloplasty (PAV). MATERIAL AND METHODS: Records of 70 patients treated with percutaneous aortic valvuloplasty with at least 6 months follow-up were reviewed. RESULTS: The 70 patients with PAV aged 3 months to 36 years, mean 10.5 +/- 10.6 years, 40) 63%) were male and 26 (37%) female. Initial systolic peak gradient decreased from 84 +/-20 to 31 +/- 16 mmHg (p < 0.05), while the reduction percentage ranged from 25 to 100%, mean 60 +/- 22. Balloon/aortic annulus index was 0.9 +/- 0.17. Ten (14.1 %) patients developed aortic insufficiency after PAV. Follow-up ranged from 6 to 168 months, mean +/- 48 months. At end of follow-up, 21 patients (30%) were considered failed cases and 49 (70%) patients had a successful outcome. CONCLUSIONS: This study showed a series with the longest follow-up in Latin America with PAV. New prospective and multicentric studies are needed in this region.

Concordance of congenital heart defects in two pairs of monozygotic twins: pulmonary stenosis and tetralogy of Fallot.

Two pairs of twins with specific concordance in congenital heart lesions are presented. We reviewed all the monozygotic twins with specific concordance in congenital heart lesions in the last 18 years. Two pairs were found. First, a pair of 13 years-old monozygotic female twins; a severe infundibular and pulmonary valve stenosis was discovered in both girls. In the second pair of twins (8 years-old boys), Tetralogy of Fallot was diagnosed by echocardiogram, cardiac catheterization and angicardiography. In fluorescence hybridization test in situ all patients were negative for microdeletion in chromosome 22q11. Anatomic differences between twins are not enough explained on genetic bases; it's necessary to consider the role of other factors, probably acquired during the first stages of embryonic development. These are the two first pairs of twins with specific concordance in congenital cardiac lesions reported in Mexico.

Insuficiencia cardiaca en niños / Heart failure in children

El objetivo de esta revisión es analizar y actualizar los aspectos más relevantes de la insuficiencia cardiaca en niños, contrastando con lo que ocurre en la insuficiencia cardiaca en adultos; esto con el propósito de resaltar los elementos comunes entre los dos grupos y, principalmente, destacar las diferencias, por ejemplo, el hecho de que la presencia de la insuficiencia cardiaca en niños no necesariamente implica el evento final de una enfermedad y que en la mayoría de estos casos el pronóstico es mejor. También se revisan en esta primera parte los aspectos históricos, las definiciones y los conceptos, así como la epidemiología, la fisiopatología y el diagnóstico.

The aim of this paper is to review and update, the most relevant aspects of heart failure in children, comparing them with heart failure in adults, in order to establish the common elements between both groups and also to emphasize the differences between both groups. For instance, heart failure in children not necessarily implied poor prognosis and in general, the prognosis in children is better. The history, concepts and definitions, epidemiology, physiopathology and clinical picture are discussed in this first part.

Cardiac pacing in balloon aortic valvuloplasty.

OBJECTIVE: To evaluate the rapid ventricular pacing in balloon aortic valvuloplasty to achieve balloon stability. MATERIAL AND METHODS: From September 2004 to July 2005, a prospective protocol was carried out: ten patients with aortic valve stenosis were treated with this method. Patient's age ranged from 3 to 16 years with mean age of 10.2+/-4.3 years. In all cases a bipolar pacing catheter was placed in the right ventricle. Rapid ventricular pacing was initiated at the rate of 150 per minute and was gradually increased to achieve a 50% drop in systemic pressure. The balloon was inflated only after the pacing rate was reached and the blood pressure dropped. Pacing was continued until the balloon was completely deflated. RESULTS: The systolic gradients across the aortic valve before balloon dilatation ranged from 40 to 110 mm Hg, mean 68.5+/-20 mm Hg. The pacing rate required to drop the pressure by 50% ranged from 170 to 250 per minute, mean 209+/-25. Balloon stability at time of inflation was achieved in all cases with no balloon movement. The post ballooning gradients ranged from 5 to 28 mm Hg, mean 19.7+/-8.3 mm Hg (p<0.001). In all cases there was no change in aortograms, performed before and after balloon dilatation in aorta, except in one patient who developed grade I aortic regurgitation. CONCLUSIONS: Rapid ventricular pacing appears to be an effective and a safe procedure to stabilize the balloon during balloon aortic valvuloplasty and is thought to decrease the incidence of aortic insufficiency.