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[Assessment of the state of pulmonary circulation in infants of the 1st year of life with complete transposition of great vessels and ventricular septal defects].

The condition of pulmonary circulation in children of the first year of life with complete transposition of the great vessels (CTGV) and interventricular septal defect (IVSD) was evaluated by comparing the complex of hemodynamic and angiometric values obtained in catheterization of the heart cavities and angiocardiography. All patients were divided into two hemodynamic groups according to the blood flow in the lungs: group 1 - with the ratio of pulmonary to systemic circulation (PC/SC) greater than 1; group 2 - with the PC/SC ratio less than 1. In group 1 patients the ratio of effective pulmonary circulation (EC) to PC is always less than the ratio of EC to SC both in normal and in increased total pulmonary resistance (TPR); in group 2 patients, in contrast, EC/PC greater than EC/SC when TPR is above 10 mu/m2. The evaluation of the degree of pulmonary hypertension in infants with CTGV and IVSD should therefore be based not only on the absolute values of PC, EC, and TPR but also on such differential-diagnostic signs as the PC/SC, EC/SC, and EC/PC ratios.

Age Factors↗

[Angiocardiographic diagnosis of ventricular septal defects in complete transposition of great vessels].

The article analyses experience in angiocardiographic diagnosis of the localization of ventricular septal defects (VSD) in 47 patients with complete transposition of the great vessels (CTGV) and high pulmonary hypertension. Axial X-ray views ("4 chambers" and "long axis") were used in the examination. The work shows the angiographic signs of all types of VSD. It is concluded that at present there is no single X-ray view universal for all types of VSD. Two-dimensional echocardiography is important in the choice of the view in angiocardiography. The muscular type of VSD is encountered most frequently (40.5%) in CTGV. Absence of contact of the superior margin of the defect with the valve of the aorta and pulmonary artery is a characteristic angiocardiographic sign of the defect. Contact of the superior margin of the defect with the valve of the pulmonary artery and absence of contact with the aortic valve are characteristics of the perimembranous types of VSD. Subarterial types of VSD are characterized by contact of the upper margin of the defect both with the aortic valve and the valve of the pulmonary artery. In 11 patients the results of angiocardiography were compared with the findings of autopsy.

Angiocardiography↗

[Diagnosis of congenital heart defects today. Part 2: Aortic stenosis, aortic isthmus stenosis, tetralogy of Fallot, transposition of great vessels].

In this second part of our review, the diagnosis of the following congenital heart disease is discussed: aortic stenosis, aortic isthmus stenosis (coarctation of the aorta), Fallot's tetralogy and transposition of the great vessels. Aortic stenosis and coarctation of the aorta each represents a spectrum of cardiac diseases of varying severity. Cases that are clinical less severe may escape diagnosis until late childhood or adolescence. Fallot's tetralogy and transposition of the great vessels in contrast, lead to cyanosis, and are therefore usually diagnosed already in the young infant. In all four conditions, the suspected diagnosis can be established on the basis of clinical or auscultatory findings. Further diagnostic clarification is achieved with the aid of non-invasive procedures such as CT scan, chest X-ray, echocardiography and, where indicated, NMR imaging. Additional cardiac catheterization is required only in the case of the tetralogy of Fallot.

Aortic Coarctation↗

[Echocardiographic evaluation of simple transposition of great vessels subjected to physiologic correction].

The aim of the present study was to evaluate the cardiovascular morphology after Senning's operation for transposition of the great vessels (TGV) using Döppler-echocardiography. The study included 25 patients (pts) aged 17 to 127 months (mean = 60 +/- 27) who were evaluated 6 to 112 months (mean = 42 +/- 27) after surgery. The following parameters were analysed: right (RV) and left ventricular (LV) morphology and function; tricuspid regurgitation and its severity; caval and pulmonary venous pathways and the presence of pulmonary stenosis, its type and severity. All the 25 pts had an abnormal end-systolic interventricular septal (IVS) configuration, with left convexity in 22 (88%) and a flat septum present in 3 (12%). The LV fractional shortening (FS) ranged from 25 to 60% (mean = 41 +/- 10) and the preejection/ejection time ratio (PET/ET) from 0.18 to 0.33 (mean = 0.24 +/- 0.04). The RV mean FS ranged from 23.0 to 32.5% (mean = 26.9 +/- 3.0) and PET/ET from 0.27 to 0.46 (mean = 0.38). The RV wall motion study revealed that the IVS was hypokinetic in 3 pts (12%), akinetic in 19 (76%) and dyskinetic in 3 (12%). Three pts also had an hypokinetic antero-apical segment. TR was detected in 22 (88%) pts being trivial in 16 (64%), mild in 4 (16%) and moderate in 2 (8%). It was always possible to visualise the neo left and right atria as well as the caval-right atrial junctions. No systemic or pulmonary venous obstruction was detected. In 3 pts there was a mild subpulmonic dynamic stenosis related with septal buldging and mitral valve apposition. In conclusion, Döppler-echocardiography can provide accurate information for the follow-up of patients with TGV submitted to physiological correction and it can reduce the number and frequency of late postoperative cardiac catheterization.

Child↗

Corrected transposition of great vessels and Ebstein's anomaly of tricuspid valve. Echocardiographic findings.

A case is reported concerning echocardiographic findings in a patient with congenital corrected transposition of the great vessels and Ebstein's anomaly of the tricuspid valve. This presented an unusual opportunity to study atrioventricular valve closure in a patient in whom the Ebstein's malformation involved the systemic atrioventricular valve. The mitral to tricuspid valve closure interval was 68 milliseconds and represented significant delay in tricuspid valve closure. This closure interval is similar to closure intervals previously reported for patients with Ebstein's anomaly without ventricular inversion.

Ebstein Anomaly↗

[Balloon (Rashkind) atrio-septostomy in transposition of great vessels in the neonatal period].

Analysis of the balloon atrial septostomy (BA) for treatment of the transposition of the great arteries (TVG) during the neonatal period will allow the evaluation of some of the changes that have occurred in the diagnosis and therapy of this congenital cardiopathy. We performed a retrospective survey of the management, evolution complications of 24 BA as a palliative technique in TGV cases, admitted to the Neonatal Intensive Care Unit during a 5 1/2 year period. The aim of the study was to evaluate the factors that indicated a bad prognosis by comparing patients that are still alive (21) with those that are dead (3). The mean age at BA performance was 4.5 +/- 5.5 days. The biological constants prior to catheterization were normal, maintaining good oxygenation, administering prostaglandins in 80% of the patients and mechanical ventilation in 25% of the patients. BA outcome was considered as good in 68%, moderate in 12% and bad in 20% of the cases. Intra-catheterization complications, mainly hemorrhages and bradycardia, were suffered by 40% of the patients and one patient died during catheterization. We could see a progressive arterial desaturation due to the lack of efficacy of the BA. As bad post-catheterization outcome factors were found: hypotension, arrhythmia, hemorrhage, greater need of dobutamine, volume expansion and mechanical ventilation. Our principal conclusion is that, although BA has improved the prognosis of newborns with TGV, because of the progressive worsening of the patients during the months following the BA, it is necessary to perform early corrective surgery.

Catheterization↗

[Angiocardiographic and echocardiographic diagnosis of complete transposition of great vessels in association with pathology of the aorta].

The article analyses experience in the diagnosis of a pathological condition of the aorta in complete transposition of the great vessels (CTGV) in infants. Pathology of the aorta was revealed in 9 (2.6%) of the 350 patients who were examined. Six patients with CTGV had coarctation of the aorta (CA), 2 had subaortic stenosis, and one patient had complete interruption of the arch of the aorta. Our experience provides evidence that two-dimensional echocardiography allows the region of the thoracic aorta to be located from a suprasternal or a high right parasternal approach. Subaortic stenosis was diagnosed in 2 patients during autopsy. Retrospective analysis of the echocardiograms and angiocardiograms revealed characteristic signs of subaortic obstruction. Absence of the continuity of the arch and descending aorta is an echocardiographic sign of interruption of the arch of the aorta which was recognized retrospectively only after angiocardiography. Right and left ventriculography must be performed for precise anatomical diagnosis of CTGV with an intact interventricular septum combined with coarctation of the aorta. In patients with concomitant interventricular septal defect this examination is supplemented by antegrade and retrograde aortography from the ascending aorta to exclude patent ductus arteriosus and for better visualization of CA. Catheterization of all heart cavities, right and left ventriculography, aortography, and pulmonary arteriography must be carried out in all patients with total interruption of the continuity of the arch of the aorta.

Angiocardiography↗