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Truncus arteriosus: successful surgical correction without the use of a valved conduit.

A new operation for the restoration of continuity between the right ventricle and pulmonary circulation in truncus arteriosus in infancy without the use of a tube graft is described. This was achieved by anastomosing the inferior margin of the detached confluent branch pulmonary arteries to the apex of a vertical infundibular ventriculotomy to form the posterior wall of the reconstructed right ventricular outflow tract. A patch of pericardium was used to form the anterior wall and complete the primary cardiac repair.

Female↗

A rare variant of common arterial trunk.

A 13 day old baby was admitted to hospital with multiple abnormalities, increasing cyanosis, and cardiac failure. Cardiac catheterisation was performed on the day of admission and he died shortly after the procedure. A rare variant of common arterial trunk, in which the pulmonary arteries arose directly from the underside of the aortic arch, was found at necropsy.

Humans↗

Endocarditis with aneurysm involving an aortic homograft used to correct a truncus arteriosus: medical-surgical salvage.

An aneurysm of an aortic homograft conduit, used to correct a type I truncus arteriosus anomaly in a four month old infant, developed when the patient was 15. Blood cultures grew Staphylococcus aureus. The aneurysm was detected by magnetic resonance imaging and digital subtraction angiocardiography. An emergency open heart operation, guided by these investigations, was performed to remove the original homograft and replace it with another valved aortic homograft. Postoperative antibiotic treatment had to be stopped when profound neutropenia developed. This responded to treatment with recombinant human granulocyte colony stimulating factor. Three years later she was symptom free and did not require medication. Chest x rays and echocardiograms showed a normally functioning heart and conduit valve.

Aortic Aneurysm↗

Rare variant of truncus arteriosus with intact ventricular septum and hypoplastic right ventricle.

A three week old girl was admitted to hospital with severe congestive heart failure and cyanosis. Cross sectional and Doppler echocardiography and cardiac catheterisation showed a unique variant of truncus arteriosus with an intact ventricular septum. The trunk rose only from the left ventricle and was associated with a hypoplastic right ventricle with sinusoids to the right coronary artery.

Cardiac Catheterization↗

Truncus arteriosus with anterior origin of a hypoplastic main pulmonary artery.

A newborn with an unusual form of truncus arteriosus is described, whose clinical presentation and certain features of her anatomy were more typical of pulmonary atresia with a ventricular septal defect. The morphological differentiation of certain types of truncus arteriosus and pulmonary atresia with ventricular septal defect remains an area of speculation among cardiac pathologists, and research in this area is being done using animal models. The anatomic features of the heart described in this report may assist in this discussion as the application of observations made in animal models to human cardiac development may be problematic.

Female↗

Di-George syndrome presenting with hypocalcaemia in adulthood: two case reports and a review.

This report describes two cases of Di-George syndrome presenting with hypoparathyroidism in adulthood. The first patient presented with profound hypocalcaemia that resulted in a generalised seizure. Routine investigations revealed hypoparathyroidism. The clue to her underlying condition was the postnatal death of her young child. This case shows that Di-George syndrome can present in adulthood with hypocalcaemia in the absence of other classic features of this condition. This has enormous implications for future family planning and may also have important health implications. The second patient, diagnosed on routine blood testing, had previously suffered with a congenital heart condition, but the syndrome was not revealed until she was of postmenopausal age. These two patients show that Di-George syndrome can present in adulthood with hypocalcaemia. This is an important observation because the condition has profound implications for health and family planning.

Adult↗

Early and late results of surgical repair of truncus arteriosus.

Ninety-two patients had corrective operation for truncus arteriosus between 1967 and 1975. During the first 30 days after surgery, 23 patients died. No significant differences appear between early mortality and sex, type of truncus, variations in pulmonary arterial anatomy, truncal valve regurgitation, associated anomalies, previous operation, or duration of extracorporeal circulation. Greater risk is probably encountered with the higher but still operable levels of pulmonary resistance. Reoperation has been required in three patients. Fifty-nine percent of survivors are symptom-free, with all but two of the remainder (38%) being in NYHA functional class II. The late result is suggestively less satisfactory in patients with significant preoperative truncal valve regurgitation. Some late complications related to deterioration of the earlier aortic homograft conduit may be avoided by use of a porcine valve Dacron conduit. The current operative mortality of 9%, and the well-being of 97% of surviving patients suggest the continued advisability of recommending operation for appropriate patients.

Adolescent↗

Truncus arteriosus with unilateral absence of a pulmonary artery. Criteria for operability and surgical results.

In 15 of 126 (12%) patients with truncus arteriosus who were catheterized at the Mayo Clinic from 1967 through 1975, natural agenesis of one pulmonary artery was present. Ten truncus patients with either natural or acquired absence of one pulmonary artery have undergone definitive operation. The criteria for operability, based on a calculation of pulmonary resistance, are different in patients with single pulmonary artery than in patients with two pulmonary arteries. Study revealed that, if the calculated pulmonary resistance in the patient with single pulmonary artery is halved, this new value provides a more reliable assessment of the reactivity of the pulmonary arteriolar bed than does the total calculated pulmonary resistance value. Follow-up information suggests that the patient with single pulmonary artery may be at potentially high risk of continued progression of pulmonary vascular disease after surgical correction, possibly because of the postoperative obligatory increased flow through the single pulmonary arteriolar bed as a result of the entire cardiac output passing through it. Surgical correction of truncus arteriosus during infancy, before significant pulmonary vascular disease has developed, appears to be particularly desirable in this subgroup of patients with single pulmonary artery.

Adolescent↗

Truncus arteriosus communis. Unusual case associated with transposition.

A child with truncus arteriosus communis, characterized by the posterior origin of an individualized pulmonary trunk is presented. This relationship between the great arteries is unusual in truncus arteriosus communis and the spatial orientation resembles that seen in transposition of the great vessels. A brief discussion is proposed about a proper terminology in this type of complex anomaly.

Child, Preschool↗

Computerized axial tomography of the chest for visualization of "absent" pulmonary arteries.

To expand the search for central pulmonary arteries in six patients with absence of cardiac-pulmonary continuity, computerized axial tomography (CAT) of the chest was performed. The CAT scans were compared with previous arteriograms and pulmonary vein wedge angiograms. Three patients with type IV truncus arteriosus were studied, and none had a central, right or left pulmonary artery on CAT scan. However, two patients with tetralogy of Fallot with pulmonary atresia and a patent ductus arteriosus to the right lung demonstrated the presence of a left pulmonary artery. In addition, one child with truncus arteriosus with "absent" left pulmonary artery demonstrated a left pulmonary artery on the CAT scan. The CAT scan may therefore enhance our ability to search for disconnected pulmonary arteries in children with complex cyanotic congenital heart disease.

Adolescent↗

Long-term survival in truncus arteriosus communis type A1 associated with Ehlers-Danlos syndrome--a case report.

Long-term survival is rare in patients with truncus arteriosus communis type A1 due to the early appearance of pulmonary artery obstructive changes. A 48-year-old woman with truncus arteriosus communis type A1 diagnosed with chest radiography, two-dimensional echocardiography, and cardiac catheterization is presented. The right ventricular pressure was 108 mm Hg, whereas the systolic pulmonary artery pressure was 98 mm Hg. A concomitant diagnosis of the Ehlers-Danlos syndrome was made with clinical findings. The association of Ehlers-Danlos syndrome, in which a collagen synthesis defect is observed in arterial vessels, might have interfered with pulmonary artery intimal proliferation, thus precluding the appearance of advanced pulmonary artery luminal obstructive changes and allowing long-term survival.

Diagnosis, Differential↗

Pulmonary vascular disease in children with congenital cardiovascular malformations. Etiologic considerations.

A detailed clinical analysis of 16 patients with various forms of congenital cardiac malformations with the potential for large left-to-right shunts who developed extreme pulmonary hypertension in infancy and early childhood has been carried out. The study plan was to include only patients with sufficiently long follow up to allow analysis of their course in both the prepubescent and postpubescent periods. In early life these patients were all characterized by lack of evidence for significant development of the expected left-to-right shunt indicating inappropriate maturation of their pulmonary vascular circulation. Throughout all phases of their life, they were significantly polycythemic in comparison with a control group but this polycythemia, representative of peripheral systemic hypoxemia, increased markedly with the acquisition of puberty in all patients. These two findings indicate both an early abnormality, suggestively primary, of the pulmonary vasculature and a late progression of the disease associated with pubertal maturation.

Adolescent↗

Hemitruncus presenting in an adult. A case report.

Hemitruncus is a rare congenital anomaly in which one pulmonary artery branch, usually the right, arises from the ascending aorta just above the aortic sinuses, whereas the main pulmonary artery and the other pulmonary branch arise in their normal position. The authors present a case in which the patient was diagnosed as having hemitruncus as an adult, and to the best of their knowledge only 7 similar cases have been reported in the medical literature.

Adult↗

Ectopic aortic origin of the right pulmonary artery in tetralogy of Fallot.

A case is described wherein Fallot's tetralogy was associated with aortic origin of the right pulmonary artery. The left pulmonary artery was the continuation of the pulmonary trunk. Although a number of examples of Fallot's tetralogy associated with aortic origin of the left pulmonary artery are known, combination of Fallot's tetralogy and aortic origin of the right pulmonary artery is rare and has been described in only two cases previously.

Angiography↗