Fate of equine pericardial roll conduit for Rastelli operation during long-term follow-up.

BACKGROUND: Right ventricular outflow tract obstruction is a frequent condition after Rastelli operation. Although several modifications have been reported elsewhere, ideal conduit has not been developed yet during long-term follow-up. We reviewed our experiences over 15-year long-term follow-up with patients who underwent Rastelli operation using house-made equine pericardial roll graft. METHODS: Since June 1981, 16 patients underwent Rastelli operation with the pericardial roll graft. Median follow-up time was 15.6 years (7.3-26.8 years). RESULTS: Twelve out of 16 patients using pericardial roll graft with (n = 6) or without (n = 6) cusps underwent 13 reoperations during the follow-up period. Median time from first Rastelli to re-do operation was 8.4 years with median time to reoperation of 8 years. Major indication for reoperation was conduit obstruction (n = 10), but not conduit regurgitation. Conduit problem includes kinking and compression of the graft. Reoperation procedures include 7 Danielson procedures, 2 patch augmentations, 1 homograft replacement, 1 pericardial roll graft, 1 expanded polytetrafluoroethylene tube graft replacement, and 1 patch closure for pulmonary artery aneurysm. Balloon angioplasty was not effective for pericardial roll conduit stenosis. CONCLUSION: We conclude that house-made equine pericardial roll graft was durable for certain time period, but conduit change may be inevitable. Because of excellent handling and wide application, further modification may be warranted.

Left pneumonectomy for lung cancer after correction of contralateral partial anomalous pulmonary venous return.

We report the successful treatment of a 48-year-old man with left lung cancer and contralateral partial anomalous pulmonary venous return (PAPVR). He was found to have an abnormal shadow on a regular checkup. Sputum cytology revealed squamous cell carcinoma. Chest computed tomography showed not only a left hilar mass but also showed that his right superior pulmonary vein was draining into the high portion of the superior vena cava. In the presence of the right partial anomalous pulmonary venous return, it was believed that left pneumonectomy would cause serious postoperative heart failure due to an increase in the left-to-right shunt. Therefore his partial anomalous pulmonary venous return was corrected first under cardiopulmonary bypass, and 3 weeks later he underwent successful radical left pneumonectomy.

[Coarctation of the aorta].

The surgical outcome of neonatal and infantile coarctation of the aorta has been improved dramatically in recent years. Not only the improvement of perioperative management based on the better understanding of the pathophysiology but the improvement of the surgical procedure made the successful outcome. There are still, however, several controversial aspects of surgical management: How to select surgical technique? How to fix the distal or transverse aortic arch hypoplasia? How to approach the intracardiac defect? How to protect spinal cord? Postoperative recoarctation in adult age will be mostly concerned in recent years. Surgeons have to aware all these short- and long-term problems when fixing this particular cardiac lesion. Now I want to discuss this not-unusual and relatively complex heart disease in this article.

Noonan syndrome associated with anomalous coronary artery and other cardiac defects.

This case-report describes an 11-month-old infant, who had been diagnosed with Noonan syndrome with a variety of associated anomalies. Multiple cardiac anomalies were present, consisting of dysplastic pulmonary valve, symmetric biventricular hypertrophy, atrial septal defect and right ventricular outflow tract (RVOT) obstruction, in which the pressure gradient measured 73 mmHg, and anomalous coronary artery. Systolic anterior motion of the mitral valve (SAM) was present, without remarkable clinical significance. RVOT transannular repair with non-cusped xenograft along with resection of hypertrophied right ventricular outflow myocardium. Left ventricular outflow tract (LVOT), which had no clinical sign of obstruction, was left untouched, expecting the RVOT repair also effectively release LVOT dynamic obstruction. The postoperative echocardiography revealed residual SAM without significant pressure gradient through LVOT.

Virilizing Adrenocortical Carcinoma Invading the Right Atrium with Histological High-Grade Malignancy and p53 Mutation in a 3-Year-Old Child: Indication of Post Operative Adjuvant Chemotherapy.

We present a 3-yr-old girl with a virilizing adrenocortical carcinoma invading into the right atrium with histological high-grade malignancy and p53 mutation. Development of facial acne and pubic hair were noted at 3 yr and 2 mo. The levels of androgens were high. Diurnal variation in ACTH and cortisol were absent. Abdominal computed tomography revealed a large right suprarenal mass, with extension into the inferior vena cava and right atrium. Based on the diagnosis of a right virilizing adrenocortical tumor with Cushing syndrome, surgery was performed by a combined thoracoabdominal approach with the patient on cardiopulmonary bypass. The tumor was 7 × 5.5 × 3.5 cm in size, and weighed 95 g. The histological diagnosis was adrenocartical carcinoma with high-grade malignancy according to the category of Weiss. A heterozygous mutation of the p53 tumor-suppressor gene (codon 248 CGC→TGG) was found. We did not perform adjuvant chemotherapy because of radical resection on macroscopic observation and no metastasis in radiological findings. Five months after the surgery, her chest X ray and computed tomography revealed multiple lung metastases and a single liver metastasis. In this type of patient with histological high-grade malignancy and p53 mutations, postoperative adjuvant chemotherapy is indicated even if macroscopic total surgical removal had been performed.

Mitral valve replacement in a 15-month-old infant with infective endocarditis.

This report describes a 15-month-old child without particular heart problems, presenting prolonged high-grade fever, an impaired level of consciousness, right hemiparesis and cutaneous lesion on admission. Medication was started according to the initial diagnosis of bacterial or viral meningitis, however, congestive heart failure was suddenly observed 15 days after the admission. Echocardiography revealed a mass in the right atrium, and mitral valve regurgitation due to the irregularly thickened and aneurysmal anterior leaflet with a perforation, consistent with infective endocarditis. Mitral valve replacement with a mechanical prosthesis was performed and the postoperative course was uneventful.

Structural differences in the cytoarchitecture and intercalated discs between the working myocardium and conduction system in the human heart.

Working and specialized cardiac myocytes and their intercalated discs in adult human hearts without history of cardiac disease were examined by scanning electron microscopy. The NaOH/ultrasonication treatment of cardiac tissues resulted in the digestion of connective tissue and separation of intercellular junctions. Auricular and ventricular working cardiac myocytes were quasi-cylindrical in shape, bifurcated, and connected end-to-end at the intercalated discs. The intercalated discs in the working cardiac myocytes showed a stair-like profile, consisting of steps (plicate segments) and corresponding risers (interplicate segments). The ventricular myocytes, in particular, had many steps and risers. The plicate segments were filled with numerous finger-like microprojections. The strands of the myocytes in the sinoatrial node were oriented linearly, while those in the atrioventricular node formed a reticular network. The intercalated discs in both nodal cells were underdeveloped, having few microprojections. Myocytes in the atrioventricular bundle (His) and the right limb were arranged in parallel, and were characterized by the presence of slender branches. Purkinje cell strands formed reticular networks. The intercalated discs in the His-Purkinje system were irregular in appearance, and the microprojections were larger in size and smaller in number than those of working myocytes. The myocytes in the crista terminalis and surrounding the fossa ovalis resembled cells in the His-Purkinje system rather than auricular working myocytes in morphology, and may act as the internodal pathway. It is concluded that morphological differences in both the cytoarchitecture and intercalated discs were closely related with contraction and impulse propagation in the various regions of the human heart.

Mortality of pulmonary artery banding in the current era: recent mortality of PA banding.

BACKGROUND: The mortality of pulmonary artery banding improved significantly in the 1980s. However, we lack information on this procedure in the current era. METHODS: The results of pulmonary artery banding in 365 patients who had operations between 1966 and 2001 were reviewed. The patients were divided into three groups: (1) group 1 patients who had operations between 1966 and 1979, (2) group 2 patients who had operations between 1980 and 1989, and (3) group 3 patients who had operations between 1990 and 2001. RESULTS: Significantly younger and smaller patients have been operated on recently (mean age: group 1, 169.0 +/- 40 days; group 2, 101.8 +/- 11 days; and group 3, 69.7 +/- 8.9 days; and mean weight: 4.6 +/- 0.1, 4.1 +/- 0.1, and 3.2 +/- 0.1 kg, respectively). A decrease was found in the number of simple cardiac anomalies, such as isolated ventricular septal defects. The early mortality in the three groups was 38.3% for group 1 (65 of 187), 13.5% for group 2 (15 of 111), and 13.8% for group 3 (12 of 87). Although the mortality did not vary significantly between groups 2 and 3, it improved over time in patients weighing less than 3 kg. Multivariate analysis of group 3 demonstrated that no isolated variable, including sex, weight, and diagnosis was a significant risk factor. CONCLUSIONS: Despite the advances in perioperative management, we found no improvement in the early mortality of pulmonary artery banding during the last decade. These results will support the preference for primary repair of intracardiac anomalies in small infants. However, this operation can now be performed with the same risk even in smaller patients. We believe that pulmonary artery banding has a role in the treatment of congenital cardiac anomalies.

As originally published in 1996. Usefulness of pulsatile bidirectional cavopulmonary shunt in high-risk Fontan patients. Updated in 2002.

BACKGROUND: A bidirectional cavopulmonary shunt has been performed for the high-risk Fontan patient. It is well known that in the presence of the bidirectional cavopulmonary shunt alone to secure pulmonary blood flow, the central pulmonary artery size decreases over time. We have performed pulsatile bidirectional cavopulmonary shunt (PBCPS), keeping pulmonary blood flow from the ventricle through the stenotic pulmonary valve, or a Blalock-Taussig shunt in patients who do not meet the criteria for the Fontan operation. METHODS: Eleven patients who underwent PBCPS between 1989 and 1993 were reviewed. We compared the results of cardiac catheterization immediately before PBCPS and during the postoperative observation period (310 +/- 257 days). RESULTS: Pulmonary blood flow and arterial oxygen saturation increased significantly after PBCPS (p = 0.01). Pumonary artery area index showed a tendency to increase (p = 0.11). The mean number of risk factors for the Fontan procedure decreased significantly from 1.8 +/- 1.1 to 0.7 +/- 0.8 after PBCPS (p < 0.05). Overall, 5 of the 11 patients (45.5%) met the criteria for the Fontan procedure, and a fenestrated Fontan procedure was carried out in 4 of them. CONCLUSIONS: The PBCPS is useful for high-risk Fontan patients not only in the staged Fontan operation, but also as definitive palliation.

Dilatable banding of a Blalock-Taussig shunt.

Dilatable banding has been used in various situations. Sometimes Blalock-Taussig shunt banding is performed to prevent pulmonary overcirculation. Recently several reports have described dilatable pulmonary artery banding. We modified these methods for flow control of a Blalock-Taussig shunt. We report the case of a neonate with truncus arteriosus in which this technique was used.

Aortopulmonary window due to balloon angioplasty after arterial switch operation.

We report 2 cases of aortopulmonary window that developed after balloon angioplasty for pulmonary artery stenosis. Both patients had undergone arterial switch operations for complete transposition of the great arteries before the angioplasty. These aortopulmonary windows were repaired through elective operations. The clinical features, diagnosis, management, and proposed mechanisms of this complication are described.