Medium-term clinical results after the Senning procedure with haemodynamic and angiographic evaluation of the venous pathways.

OBJECTIVE: To assess the clinical condition of patients and the adequacy of their newly constructed venous pathways after the Senning operation for simple transposition of the great arteries. PATIENTS AND DESIGN: All 34 patients who had the Senning operation between March 1983 and December 1986 were reviewed. Survivors had detailed cardiac catheterisation studies one to two years later and clinical evaluation two to six years after surgery. RESULTS: There were two operative deaths (6%), one sudden late death (after two years), and 31 survivors (91%). No clinical evidence of obstructed venous pathways was found and there was no need for reoperation for any reason. The average mean (SD) pressure gradient at the junction of the superior vena cava and systemic venous atrium was 2.0 (1.7) mm Hg, although two patients had gradients of 7 mm Hg. The average gradient was 0.7 (1) mm Hg in the inferior vena caval pathway, and 1.4 (1.1) mm Hg between the mean pulmonary arterial wedge and pulmonary venous atrial pressures. Only the two patients with gradients of 7 mm Hg at the junction of the superior vena cava and the systemic venous atrium had considerable narrowing of the pathway and retrograde flow in the azygos vein to below the diaphragm. CONCLUSION: A small series of patients were comprehensively studied after the Senning operation for simple transposition of the great arteries. Scrupulous technique in the construction of the venous pathways has had excellent results with no need for reoperation for obstruction in either the systemic or pulmonary pathways. Clinical observation alone may fail to identify patients with venous pathway obstruction, therefore careful assessment is required, even in patients who are physically normal.

Patterns of ductal tissue in coarctation of the aorta in the first three months of life.

A possible relationship between recurrence of coarctation and the presence of residual ductal tissue in the aorta was studied by defining the presence and pattern of ductal tissue in aortic segments from 23 patients less than 3 months of age who underwent resection of coarctation of the aorta. The surgical policy was to perform extensive excision of the coarctation, including a wide margin of descending aorta beyond the ductus arteriosus. Histologic examination showed that there was a circumferential sling of ductal tissue extending from the ductus arteriosus and surrounding the aorta at the level of the coarctation shelf in 22 specimens. In 15 of these specimens one or two tonguelike prolongations of ductal tissue extended distally from the circumferential sling and occupied constant positions in the aortic wall; one tonguelike prolongation extended distally below the insertion of the ductus and the other from the circumferential sling opposite the ductal insertion. Incomplete excision of ductal tissue was found in 11 specimens. In relation to the goal of avoiding recurrent coarctation after repair in the early months of life, the possible implications of these findings are discussed, and, in particular, a possible mechanism of recurrence after subclavian flap aortoplasty is proposed.

Distal aortic pressure during coarctation operation.

Mean distal aortic pressure (DAP) was monitored continuously during operative repair of coarctation of the aorta in 67 children more than 1 year of age between 1982 and 1987. At initial test clamping a DAP of 45 mm Hg or more was considered adequate. In 42 patients (group A), DAP exceeded 45 mm Hg and the operation proceeded. In 25 patients (group B) the DAP was less than 45 mm Hg, and adjustments were made to the position of the proximal and distal clamps and in the use of hypotensive agents. After these adjustments in group B, DAP rose from 34.7 to 50.6 mm Hg (p less than 0.001), achieving the desired level in all but 5 patients, who required temporary shunts to support the distal circulation. The adjustments were as follows: the left subclavian artery was open in 28% of patients before and 60% after (p less than 0.02); no more than one pair of intercostal arteries was clamped in 64% before and 88% after (p less than 0.05); and no hypotensive agents were used in 56% before and 80% after (p = 0.07). During the first ten minutes of cross-clamping the DAP rose by 5.5 mm Hg (p less than 0.01, n = 52). It is concluded that continuous monitoring of DAP contributed to the operative management of these patients by indicating when adjustments in the position of the clamps or in the use of hypotensive agents were needed. The use of hypotensive agents should be reduced to achieve a higher DAP.

Management of severe chest injuries.

Anoxia and haemorrhage are the causes of early death in patients with severe chest injuries. Early recognition with prompt resuscitation is the cornerstone of their management. Intensive care and a cohesive multidisciplinary approach to patients with associated multiple injuries are important.

Coexistent cardiac tumours and malformations of the heart.

Cardiac tumours and anatomical malformations of the heart may produce similar clinical signs and symptoms. The coexistence of these two abnormalities complicates diagnosis and probably adversely affects prognosis. We present a review of four cases of this rare combination. In the first case, Ebstein's malformation was present in a child with tuberous sclerosis and cardiac rhabdomyomata. Right ventricular rhabdomyomata were associated with a hypoplastic tricuspid valve in the second case. In the third case, cardiac myxomas were detected in a child with a double-chambered right ventricle. The fourth case was a child with a fibroma of the right ventricle with pulmonary atresia. We propose that, in some circumstances, a space-occupying lesion may be associated with, or possibly induce, a malformation within the developing heart.

Aspergillus ball thrombus occluding a homograft conduit.

Almost total occlusion of an aortic homograft conduit by a ball of Aspergillus terreus was found postmortem in a 6-month-old infant following repair of a type I truncus arteriosus. Signs of pulmonary hypertensive crisis, fever, anaemia, and thrombocytopenia were present. However, blood cultures were persistently negative. A 2-D echocardiogram failed to demonstrate any vegetation. The clinical, pathological, and postmortem findings are described and difficulties in the diagnosis of Aspergillus infection are discussed.

Long-term results of the "palliative" Mustard operation.

A long-term follow-up study after 41 palliative Mustard operations for transposition of the great arteries and pulmonary vascular obstructive disease is presented. The operations were performed from 1973 to 1980. Mean pulmonary arteriolar resistance was 13.96 +/- 6.7 Um2. A ventricular septal defect was not closed in 34 patients; in 7 it was created at operation. There were three hospital and two late deaths. Survivors were followed up for 3 to 10 years (mean 76.7 +/- 22.8 months). Before operation 4 children were in New York Heart Association functional class IV and 33 were in class III. Mean hemoglobin concentration was 19.43 +/- 3.14 g/dl and arterial oxygen saturation was 63.44 +/- 11.29%. After operation 18 patients were in functional class I, 17 in class II and only 1 in class III. Hemoglobin level decreased to 14.19 +/- 2.3 g/dl and arterial oxygen saturation improved to 89.12 +/- 7.25%. Cardiac catheterization in 21 survivors confirmed no change in pulmonary artery pressure and resistance. Effective pulmonary blood flow improved from 1.39 +/- 0.39 before to 2.6 +/- 0.78 liters/min per m2 after operation. The palliative Mustard operation carries a low risk and provides excellent symptomatic improvement up to 10 years in patients with transposition of the great arteries and pulmonary vascular disease.

Effect of adenosine deaminase inhibitors on the heart's functional and biochemical recovery from ischemia: a study utilizing the isolated rat heart adapted to 31P nuclear magnetic resonance.

The concept of limiting irreversible damage due to ischemic arrest by inhibiting nucleoside breakdown was tested in the isolated perfused rat heart. Functional recovery measurements were combined with continuous high-energy phosphate measurements by means of 31P nuclear magnetic resonance (NMR) and with nucleoside release measurements in the reperfusion period. The adenosine deaminase inhibitors erythro-9-(2-hydroxy-3-nonyl) adenine (EHNA) and 2'-deoxycoformycin (DCF) were given 5 min before ischemia and for the first 5 min of reperfusion. These treated groups were compared with a control, untreated group. These were further compared with a group of hearts arrested with potassium and to a group combining potassium arrest and EHNA. It was found that all treated groups recovered mechanical function significantly better than the untreated group. DCF, K+, and K+ + EHNA slowed ATP decline and resulted in better ATP recovery than untreated or EHNA-treated, and all treatments decreased nucleoside base release. Intracellular pH fell equally in all groups and recovered to preischemic values. Thus, these adenosine deaminase inhibitors improve functional recovery following ischemia, although this improvement was not well correlated with purine losses observed during reperfusion.

Factors associated with periprosthetic leakage following primary mitral valve replacement: with special consideration of the suture technique.

Among 435 patients without native valve endocarditis who were followed up to 69 months after primary mitral valve replacement, 25 developed documented periprosthetic leakage. In 10 patients this was associated with prosthetic valve infection. No evidence of prosthetic infection was found in the remaining 15 patients with documented leakages, and they form the basis of the study. Multivariate Cox regression analysis indicated that leakage in the absence of infection was strongly associated with the use of small monofilament suture (2-0 or 3-0 versus 1-0) in a continuous suture technique (92.0% actuarially leak free by 43 months versus 99.0% for continuous 1-0 monofilament sutures or pledgeted mattress sutures; p = 0.01) and with annular calcification (p = 0.01). We did not find (p greater than 0.2) the functional type of mitral valve lesion or its pathology, or the type and size of prosthesis used, to be incremental risk factors.

A case of false aneurysm of the right ventricle.

False aneurysms of the right ventricle are rare and potentially fatal. A patient is described in whom a trilocular false aneurysm arose from the antero-inferior border of the right ventricle and entered the left hemi-diaphragm. It was successfully treated by closure of the communication with the right ventricle and relief of pulmonary stenosis with right ventricular outflow tract reconstruction. The fifteen previously reported cases of false aneurysms of the right ventricle are reviewed.