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PubMed1986

Anomalous origin and malposition of the pulmonary arteries (crisscross pulmonary arteries) associated with complex congenital heart disease.

Anomalous origin and malposition of the branch pulmonary arteries is an uncommon malformation that has previously been noted only at the time of pathologic examination. Two infants with complex congenital cardiac disease had malposition of the branch pulmonary arteries (crisscross pulmonary arteries) detected by angiography and confirmed during surgery and in one case, at autopsy. The presence of this clinically unsuspected lesion complicated the accurate interpretation of both the two-dimensional echocardiographic appearance of the great vessels, and the catheter course and location during cardiac catheterization. Angiography clearly defined the crisscrossing pattern of the branch pulmonary arteries. The recognition of this malformation was important in planning and executing the surgical palliation or repair for these infants.

Electrocardiography↗
PubMed1985

Congenital heart disease in infancy--surgery for specific defects.

The evolution of surgery for congenital cardiac malformations in infancy in the past decade warrants an analysis of the indications, technical aspects, advantages, complications and results of the treatment of specific defects. Considerable variations in approach are found, and every surgeon should attempt to evaluate the most appropriate current management in each particular case. Primary closure is usually preferred to pulmonary artery banding in patients with large ventricular septal defects. But will the same approach produce a high late survival rate in patients with other left-to-right shunts, e.g. atrioventricular canal and truncus arteriosus? Primary repair of tetralogy of Fallot, for example, especially in patients who might require a pulmonary outflow tract patch, has been challenged by many. In which patients, if any, is an initial systemic-pulmonary anastomosis the preferable treatment? Which is the best surgical approach for other cyanotic congenital cardiac malformations such as transposition of the great arteries and pulmonary and tricuspid atresia? The multitude of procedures available for 'complete correction' of some malformations is an indication of their limitations, while other defects, such as some forms of univentricular heart and hypoplastic left heart, are not yet fully correctable.

Aortic Valve Stenosis↗
PubMed1985

The teratogenic effects of a bis(dichloroacetyl)diamine on hamster embryos. Aortic arch anomalies and the pathogenesis of the DiGeorge syndrome.

A study of the teratogenic effects of Fertilysin, a bis(dichloroacetyl)diamine, on hamster embryos was undertaken for a comparison of Fertilysin-induced malformations with the DiGeorge syndrome of human patients, a rare birth defect characterized by thymic and parathyroid gland agenesis, abnormal facies, and aortic arch malformations. In treated hamsters, malformations of the aortic arches were found to be consistently produced, and many of these anomalies could not be explained by conventional methods of analysis. A pathogenetic scheme to classify malformations of the aortic arch and great vessels is offered, including a revision of Celoria and Patton's classification of interrupted aortic arches. It is suggested that the DiGeorge syndrome is a syndrome of truncated pharyngeal arch development with two general forms: 1) agenesis of or incomplete development of the sixth pharyngeal arch with pulmonic circulation through the fifth arch; 2) agenesis of or incomplete development of the fifth and sixth pharyngeal arches with pulmonic circulation through the fourth arch. The third aortic arch may descend into the thorax under these circumstances.

Abnormalities, Drug-Induced↗
PubMed1985

Fifteen-year experience with surgical repair of truncus arteriosus.

From 1965 until March, 1982, 167 patients underwent surgical repair of truncus arteriosus. The age at operation ranged from 18 days to 33 years (mean 6 years). There were 48 hospital deaths (28.7%). The following factors had a positive correlation with the possibility of a surgical death: age at operation less than 2 years (p less than 0.001), a postrepair pulmonary arterial/left ventricular pressure ratio greater than 0.5 for patients with two pulmonary arteries (p less than 0.001) and greater than 0.6 for patients with unilateral absence of a pulmonary artery (p less than 0.02), and a postrepair right ventricular/left ventricular pressure ratio greater than 0.8 (p less than 0.008). The 119 hospital survivors were followed up for a total of 829 person-years. Late survival rate at 5 years was 84.4% and at 10 years, 68.8%. Preoperative factors that correlated with a reduced long-term survival rate were as follows: increasing age at operation (p = 0.004), the presence of moderate or severe truncal valve insufficiency (p = 0.008), lower pulmonary/systemic flow ratio (p = 0.04), and unilateral absence of a pulmonary artery (p less than 0.001). Thirty-six patients required reoperation during the follow-up period (30%) primarily for replacement of the right ventricular-pulmonary arterial conduit and/or for truncal valve replacement. The long-term results obtained in these patients support the need for early repair of the anomaly, improvement in the methods for control or repair of the truncal valve insufficiency, and the continued search for better extracardiac valved conduits.

Adolescent↗
PubMed1979

[Diagnostic of left-to-right shunt by isotopic angiocardiography (author's transl)].

Isotopic angiocardiography was performed in 60 patients with proven congenital left-to-right shunt. A 99mTc labeled bolus was injected in a peripheral vein, and the first passage in the heart cavities was studied. Time-activity curves of the cardiac chambers were analysed, using a scintillation camera and a data processing system. In our study, we were able to confirm the presence of the shunt in 77% of the cases in which the radioactive bolus arrived well grouped in the vena cava. Quantitative results however remain imprecise. Isotopic angiocardiography indications are compared to radiologic angiocardiography and to right heart catheterism indications. Use of this non invasive technique could render more aggressive examinations unnecessary.

Adult↗