Duplicidad del segmento postrenal de vena cava inferior (VCI) asociada a ausencia del segmento hepático / Post-renal segment duplicity of the interior vena cava (IVC) associated to absence of hepatic segment

Se describe un caso de anomalía anatómica del sistema venoso sistémico consistente en la duplicidad de vena cava inferior en su segmento postrenal asociada a ausencia del segmento hepático. Se trata de una paciente de 13 años remitida a nuestro servicio por soplo cardiaco catalogado de funcional, asintomática, con sospecha radiológica de continuidad cava-ácigos no confirmada ecocardiográficamente a la que finalmente se realizó un estudio mediante resonancia magnética (R.M) de corazón y grandes vasos encontrándose doble segmento postrenal de VCI y ausencia del segmento hepático de la misma con continuidad ácigos-cava superior (AU)

A case of anatomic abnormality of the systemic venous system consisting in duplicity of the inferior vena cava in its postrenal segment associated to absence of the hepatic segment is described. This is a 13 year old patient referred to our service due to heart murmur classified as functional, asymptomatic, with X-ray suspicion of azygo-caval continuity not confirmed on the echocardiography. Finally a Magnetic Resonance Imaging (MRI) study was made of the heart and large vessels, finding double postrenal segment of the IVC and absence of hepatic segment of it with superior azygo-caval continuity (AU)

[Balloon pulmonary valvuloplasty in the neonatal period. The clinical and echocardiographic effects]. / Valvuloplastia pulmonar con balón en el período neonatal. Efectos clínicos y ecocardiográficos.

AIM: To analyze the efficacy of balloon pulmonary valvuloplasty (BPV) as the elective treatment for neonatal critical pulmonary valvar stenosis (PVS). MATERIALS AND METHODS: The results of clinical and echocardiographic features before and after the BPV were reviewed in 29 neonates (18+/-12 days of life). Different hemodynamic and 2-D color Doppler echocardiographic were evaluated. The BPV result was classified as favourable if no other balloon or surgical therapy was required to normalise pulmonary flow and achieve a sustained right ventricle-pulmonary artery (RV-PA) Doppler gradient below 40 mmHg. It was considered unfavourable if the neonate died, needed surgery or redilation and/or the RV-PA Doppler gradient was > or =40 mm Hg. The study developed in three phases: pre BPV immediate post BPV until the hospital discharge (14+/-11 days), and in the mid-term follow-up of between 8 and 96 months (51+/-31 months). RESULTS: Mortality was not registered with BPV. The RV/left ventricular systolic pressure decreased from 1.4+/-0.3 to 0.8+/-0.3 (p<0.01) as a consequence of the dilation, and the the systemic oxygen saturation increased from 85 +/-12 to 92+/-6% (p<0.01). The RV-PA Doppler gradient diminished from 86+/-18 to 28+/-16 mm Hg immediately after BPV (p<0.01) and was registered at 13+/-6 mm Hg in the follow-up (n = 24). The RV-PA junction Z value grew from -1.25+/-0.9 before valvuloplasty to -0.51 +/-0.7 at the final echocardiogram (p<0.01). No changes in the tricuspid diameter were detected between both periods of time. Five neonates obtained unsatisfactory results: 4 in the immediate post BPV (systemic-pulmonary artery shunt 2, transannular patch 2), and 1 in the mid-term follow-up (valvectomy + transannular patch). The actuarial curve reflects that 82,7% of the patients were free form reinterventions at 8 years. CONCLUSIONS: BPV is safe and effective to relief PVS in the neonate. The balloon promotes advantageous changes in both, pulmonary annulus and the right ventricle. In addition, the RV-PA Doppler gradient observations in the follow-up, support the expectation that the BPV is a "curative" therapy.

[Left heart catheterization by the transseptal route]. / Cateterismo cardíaco esquerdo por via transeptal.

PURPOSE: To analyse the transseptal catheterization technique, its indications and possible complications. METHODS: The transseptal catheterization has been undertaken in 233 patients, 202 children (aged 0.1 to 16 years) with congenital heart disease and 31 adults (aged 37 to 73 years) submitted to mitral valvuloplasty. The Mullins technique was employed to access left heart chambers. RESULTS: In the pediatric group, the indications for transseptal catheterization were coarctation of the aorta and valvular or subvalvular aortic stenosis. By this technique, multiple diagnostic analysis and therapeutic procedures, such as blade atrioseptostomy and mitral valvuloplasty, became available. Among the complications in the 2 groups, pericardial perforation was the most frequent, depending on the laboratory practice with this technique. CONCLUSION: The transseptal cardiac catheterization is a safe and effective technique to investigate hemodynamic data in several congenital heart diseases and is essential to many therapeutic procedures. The technique has a low incidence of complications when employed by experienced teams.

[Isolated ventricular inversion associated with complete atrioventricular septal defect with total anomalous pulmonary venous drainage]. / Inversión ventricular aislada asociada a defecto completo del septo atrioventricular con drenaje venoso pulmonar anómalo total.

Based upon the description of the clinico-pathological characteristic of a case with isolated ventricular inversion plus other cardiac malformations, the clinical, diagnostic and embryo-pathological implications of this entity are reviewed.

[Surgery of congenital cardiopathies without previous catheterization. Preoperative evaluation and surgical indication using bidimensional echocardiography and Doppler]. / Cirugía de las cardiopatías congénitas sin cateterismo previo. Valoración preoperatoria e indicación quirúrgica por ecocardiografía bidimensional y Doppler.

With the non-invasive devices available nowadays, many congenital heart diseases may be diagnosed with a definition comparable and even perhaps superior to catheterization and angiography. According to this issue, from January 1983 to December 1987, 239 children underwent operations for palliation or correction of different cardiopathies based on an echo-Doppler assessment without prior catheterization. Preoperative echo-Doppler diagnoses had an adequate correlation with the surgical findings in 234 cases (97.9 percent), so there was no correlation in 5 cases. In general, we consider that echo-Doppler provides a complete and definitive diagnosis in most cases, thus eliminating the need for further invasive procedures, although we still have to rely on catheterization to obtain the necessary information or reassurance in some cases. Progressive increase of reliance in echo-Doppler techniques by the surgeon and self confidence of the cardiologist in his accurate state of the art will considerably increase the performance of cardiac operations without prior catheterization in the future. At present, this is our current policy, as day after day, we are increasing the diagnoses in which we consider unnecessary the catheterization to indicate surgery.

[Atrioventricular septal defect with associated tetralogy of Fallot. Clinico-morphological aspects and surgical considerations]. / Defecto del septo atrioventricular con tetralogía de Fallot asociada. Aspectos clinicomorfológicos y consideraciones quirúrgicas.

In this work the clinical features, diagnostic methods, surgical procedures and postoperative follow-up are reviewed in 19 cases of atrioventricular septal defect with associated tetralogy of Fallot. The clinical situation was always related to the degree of pulmonary ischemia, that pointed out the urgency and need for a palliative surgical procedure before corrective surgery. There was no data in the ECG for the differential diagnosis of isolated forms of atrioventricular septal defect. In 50% of the cases the cardio-thoracic index was increased by right atrial enlargement. The diagnosis of the malformation was made in every case by means of 2D echocardiography. In 16 cases palliative surgery was done (aortic-pulmonary shunts), with one death (6.2%) due to facts unrelated to the technique. Of the 6 cases with corrective surgery (5 cases with previous shunts), one needed a mitral valve replacement (St Jude prosthesis). There was no mortality in this group and the functional status in the long-term follow-up was: 3 cases were in the functional class I and 3 cases in class II. The overall mortality for all surgical procedures carried out was 4.3%. In conclusion, we consider the use of the corrective surgery adequate to the clinical stability of the patients with atrioventricular septal defect with associated Fallot's tetralogy.

Congenital mitral valve anomalies in transposition of the great arteries.

Seventy-nine specimens with transposition of the great arteries were reviewed. Among them were 45 with ventricular septal defect, including 6 with so-called "posterior aorta transposition". The morphology of the mitral valve was carefully studied. Mitral valve anomalies were found in 24 cases (30%). The anomalies involving the mitral valve were classified into 4 groups according to the different components of the valve: A) specimens with anomalies of the leaflets--2 cases with mitral cleft; B) those with anomalies of the commissures--1 case with a mitral gap; C) those with abnormalities of the chordae tendinae--2 cases of malattachment of the chordae tendinae to the ventricular septum and 2 with bridging chordae between the papillary muscles; and D) those with abnormalities of the papillary muscles--5 cases with a parachute valve, 10 with a "form fruste" of the parachute mitral valve (hypoplasia of one papillary muscle and short chordae tendinae) and 2 with abnormally placed papillary muscles, which reached the pulmonary annulus. In 14 cases, abnormalities of the tricuspid valve were also encountered, including 8 cases with a tricuspid gap and 6 with a malattachment of tricuspid chordae on the border of a ventricular septal defect. It can be concluded that mitral valve anomalies are not uncommon in transposition of the great arteries. This pathology must be carefully evaluated at the time of surgery, particularly in those patients for whom an anatomic correction is considered, for in those cases the left ventricle will be supplying the systemic circulation and therefore, a completely normal mitral valve will be required.(ABSTRACT TRUNCATED AT 250 WORDS)

Rebdomioma intracardíaco: procedimientos, diagnósticos y tratamiento / Intracardiac rhabdomyoma: diagnostic and treatment

Presentamos 12 casos de rabdomioma intracardíaco. Revisamos la edad, formas clínicas de presentación y los hallazgos en las exploraciones complementarias. El diagnóstico de tumor intracardíaco se hizo por ecocardiografía en 11 casos; un caso con afectación miocárdica difusa fue diagnosticado inicialmente de miocardiopatía hipertrófica. Se indicó tratamiento quirúrgico en 8 casos, por presentar distintas formas de obstrucción al flujo sanguíneo intracardíaco, realizándose en 7. El diagnóstico de rabdomioma se confirmó en 9 casos por estudio histológico. De los 12 casos viven actualmente 8, 5 operados y 3 sin operar, todos asintomáticos cardiológicamente; 5 (62%) presentan manifestaciones neurocutáneas de esclerosis tuberosa

Rebdomioma intracardíaco: procedimientos, diagnósticos y tratamiento / Intracardiac rhabdomyoma: diagnostic and treatment

Presentamos 12 casos de rabdomioma intracardíaco. Revisamos la edad, formas clínicas de presentación y los hallazgos en las exploraciones complementarias. El diagnóstico de tumor intracardíaco se hizo por ecocardiografía en 11 casos; un caso con afectación miocárdica difusa fue diagnosticado inicialmente de miocardiopatía hipertrófica. Se indicó tratamiento quirúrgico en 8 casos, por presentar distintas formas de obstrucción al flujo sanguíneo intracardíaco, realizándose en 7. El diagnóstico de rabdomioma se confirmó en 9 casos por estudio histológico. De los 12 casos viven actualmente 8, 5 operados y 3 sin operar, todos asintomáticos cardiológicamente; 5 (62%) presentan manifestaciones neurocutáneas de esclerosis tuberosa (AU)

Brucella endocarditis on double valvular prosthesis.

The case is reported of a 48-year-old man suffering from Brucella endocarditis on a double prosthesis. The successful medical and surgical treatment is described. So far as the authors know, this is the first report of Brucella endocarditis from a heart valve prosthesis.