Primary definitive repair of type B interrupted aortic arch, ventricular septal defect, and patient ductus arteriosus. Early and late results.

Since March, 1974, eight patients, aged 7 days to 5 months, with type B interrupted aortic arch (IAA), ventricular septal defect (VSD), and patent ductus arteriosus (PDA) were treated at the Columbus-Presbyterian Medical Center and the University of Maryland Hospital. Six of these patients underwent definitive repair utilizing deep hypothermia and circulatory arrest. Correction involved resection of all ductal tissue, primary anastomosis of the aortic arch, closure of the foramen ovale, and patch closure of the VSD. In five patients, all arch vessels were preserved and no prosthetic material was used to reconstruct the aortic arch. One patient died 48 hours postoperatively of a coagulopathy. All others survived more than 30 days. One patient, 3 1/2 months old at repair, had undergone pulmonary artery banding at another institution at 11 days of age; he died of recurrent respiratory infections 8 months after correction. Three patients are alive and well 3 to 6 years after repair. Two have undergone repeat cardiac catheterization which demonstrated good growth of the anastomosis and no residual gradient. Primary definitive correction of type B IAA with VSD and PDA provides distinct advantages over palliative or other surgical procedures with excellent long-term results.

Pulmonary atresia with intact ventricular septum. Sixteen-year experience.

Infants with pulmonary atresia and intact ventricular septum (PA-IVS) usually require urgent surgical intervention. Thirty patients with this anomaly, seen at the Columbia-Presbyterian Medical Center between 1962 and 1978, had palliative operations, 26 within the first 3 days of life. Six underwent a closed pulmonary valvotomy alone, with no survivors; six had only a systemic--pulmonary artery shunt, with three early survivors. Because of this experience, 17 had a combined procedure of valvotomy and shunt, with 14 early survivors. One patient recently underwent a definitive right ventricular outflow patch procedure with cardiopulmonary bypass. Eight patients subsequently have had corrective open-heart procedures, with five patients surviving from 2 to 10 years. A unicusp aortic homograft was used for repair in five and a Hancock valved conduit in three. Four patients are presently awaiting operation. We conclude that the initial surgical management of these critically ill infants must not only increase pulmonary blood flow but in addition provide an opportunity for right ventricular growth. Thus we continue to advocate the combined procedure of a valvotomy plus a shunt to provide adequate palliation. Repeat catheterization should be performed within a year to confirm the adequacy of the valvotomy, since this is essential to maximal right ventricular enlargement and to allow for definitive correction at a later date.

Pulmonary resection for invasive Aspergillus infections in immunocompromised patients.

Standard antifungal medical therapy of invasive pulmonary aspergillosis that occurs in immunocompromised patients with hematologic diseases with neutropenia or in liver transplant recipients results in less than a 5% survival. In view of these dismal mortality rates, we adopted an aggressive approach with resection of the involved area of lung along with systemic antifungal therapy when localized invasive pulmonary aspergillosis developed in these patients. Between January 1987 and December 1993, 14 patients with hematologic diseases and 2 liver transplant recipients underwent resection of acute localized pulmonary masses suggestive of invasive pulmonary aspergillosis a median of 7.5 days (range 1 to 45 days) after the diagnosis was clinically suggested and confirmed by chest computed tomographic scans. Operative procedures done included two pneumonectomies, one bilobectomy with limited thoracoplasty, nine lobectomies, and five wedge resections (one patient with hematologic disease had two procedures). All patients were treated before and after the operation with antifungal agents. Nine (64%) of 14 patients with hematologic disease and 2 (100%) of 2 liver transplant recipients survived the hospitalization with no evidence of recurrent Aspergillus infection after a median 8 months of follow-up (range 3 to 82 months). The five hospital deaths (all patients with hematologic diseases) occurred a median of 20 days after operation from diffuse alveolar hemorrhage in three, graft-versus-host disease in one, and multiple organ system failure with presumed disseminated Aspergillus infection in one. Four of the five deaths were in patients with allogeneic bone marrow transplants. Two of the three patients requiring resection of multiple foci of infection died, as did the only patient who was preoperatively ventilator dependent. In immunocompromised patients with hematologic diseases or liver transplantation with invasive pulmonary aspergillosis, early pulmonary resection should be strongly considered when the characteristic clinical and radiographic pictures appear.

Subclavian flap repair of coarctation of the aorta in neonates. Realization of growth potential?

The subclavian flap repair for coarctation of the aorta allows potential for growth by utilizing autogenous tissue. Although well documented in young children, its promise in the tiny neonate warrants further evaluation. Since August, 1979, 29 patients, including 24 infants, have undergone subclavian flap repair at the University of Maryland Hospital. Weights ranged from 1.4 to 5 kg (mean 3.2 kg). All patients less than 6 months old had associated intracardiac defects and were in severe congestive failure. Fifteen responded to preoperative prostaglandin infusions. The overall early mortality was 14%; among the neonates it was 21%; and among those operated upon within the first week of life, 33%. There was one intraoperative death among the eight patients who underwent simultaneous pulmonary artery banding. There were no deaths among patients older than 5 days at operation. Four of the five neonates who died had some variant of hypoplastic left heart syndrome, with severe stenosis or atresia of the systemic atrioventricular valve, critical aortic stenosis, or hypoplastic left ventricle. Twenty-two survivors continue to do well up to 3.7 years postoperatively (mean follow-up 26 months). At follow-up all patients are normotensive with brisk lower extremity pulses. Patients now weigh 1.3 to 6.9 (mean 2.3) times their operative weight, and only one patient has a measured arm-to-leg gradient greater than 10 mm Hg (mean gradient 3.7 mm Hg). Seven of the neonates have undergone repeat catheterization, and all had satisfactory growth of the subclavian flap segment of repair and no gradient. Two older patients (3 and 4 years old at operation) have undergone exercise testing 3.7 years after repair, with peak exercise gradients of only 7 and 15 mm Hg. We therefore continue to utilize this technique for the treatment of coarctation even in tiny neonates.

Valve replacement in the right side of the heart in children: long-term follow-up.

Twenty-five patients (16 male, 9 female) underwent right-sided valve replacement (10 pulmonary valve replacement, 14 tricuspid valve replacement, 3 tricuspid plus pulmonary valve replacement, and 2 replacements of a single atrioventricular valve) at the University of Nebraska Medical Center from June 1977 to December 1986. Twenty-one patients (84%) are long-term survivors with 2,035 months follow-up (range, 41 to 143 months; mean, 96.9 months). Twenty-three Carpentier-Edwards bioprosthetic valves, one Ionescu-Shiley bioprosthetic valve, and nine St. Jude Medical valves were inserted. Follow-up of 17 patients with a Carpentier-Edwards valve ranged from 5 years 9 months to 11 years 9 months (mean, 8 years 11 months). To date there has been one reoperation after 3 years 4 months in this group. One patient who received an Ionescu-Shiley bioprosthesis required re-replacement at 20 months after operation. Three of 4 patients who received St. Jude mechanical valves and are long-term survivors have required replacement after 36 to 56 months. We conclude that the Carpentier-Edwards bioprosthetic valve is a viable option in the right side of the heart in the young age group when annular size is adequate to accommodate an appropriate bioprosthesis.

Intrapleural fibrinolytic treatment of multiloculated thoracic empyemas.

Acute multiloculated thoracic empyemas incompletely drained by tube thoracostomy alone usually require operation. To avoid a thoracotomy yet treat this difficult problem, intrapleural fibrinolytic agents were employed. Between April 1, 1990, and April 1, 1993, 13 consecutive patients presenting with a fibrinopurulent empyema were demonstrated to have incomplete drainage. To facilitate drainage, streptokinase, 250,000 units in 100 mL 0.9% saline solution (3 patients), or urokinase, 100,000 units in 100 mL 0.9% saline solution (10 patients), was instilled daily into the chest tube, and the tube was clamped for 6 to 12 hours followed by suction. This routine was continued daily for a mean of 6.8 +/- 3.7 days (range, 1 to 14 days) until resolution of the pleural fluid collection was demonstrated by computed chest tomography and clinical indications. This regimen was completely successful in 10 of 13 patients (77%), who had resolution of the empyema, eventual withdrawal of chest tubes, and no recurrence. Two patients, both pediatric liver transplant patients, had an initial good response but eventually required decortication. One patient with a good radiographic response became increasingly febrile during streptokinase therapy and underwent a thoracotomy, but no significant undrained fluid was found. This patient's continued fever was believed to be a streptokinase reaction. Urokinase was used subsequently. No treatment-related mortalities or complications occurred. Intrapleural fibrinolytic agents, especially urokinase, are safe, cost-effective means of facilitating complete chest tube drainage, thereby avoiding the morbidity of a major thoracotomy for 77% of a group of multiloculated empyema patients who traditionally would have required open surgical therapy.

Management of penetrating cardiac injuries: the role of emergency room thoracotomy.

Sixty-four consecutive patients with penetrating cardiac injuries were treated between January, 1977, and January, 1983, at the University of Maryland Hospital. Twenty-eight patients had major associated injuries of other organs. The patients were divided into groups according to their clinical status on arrival. An aggressive approach was utilized including early emergency room (ER) thoracotomy for "lifeless" or deteriorating patients. Three patients required immediate cardiopulmonary bypass for repair of their injuries. Twenty-one (57%) of the 37 patients undergoing ER thoracotomy survived; most of the deaths occurred in patients arriving "lifeless" from gunshot wounds. Twenty-four (89%) of the 27 patients who were in stable enough condition to undergo initial repair in the operating room (OR) survived. Overall survival was 45 patients (70%). Though superficial wound infections developed in 18 patients, there were no deep or systemic infections. None of the survivors sustained severe neurological sequelae. Five patients underwent late reoperations for closure of a ventricular septal defect (2), mitral valve replacement (1), and pericardiectomy (2) with no deaths. Though repair of penetrating cardiac injuries should preferably be carried out in the OR, immediate thoracotomy for "lifeless" or deteriorating patients can be performed in the ER with a low incidence of direct surgical complications and with high patient survival.

Structural failure of a Starr-Edwards aortic track valve.

Structural failure of a Model 2400 Starr-Edwards aortic track valve occurred suddenly, 4 years after implantation. At operation, the valve cage was removed from the descending aorta. Examination of the excised prosthesis disclosed minimal cloth wear and no evidence of infective growth; however, three struts were fractured above their insertion into the valve ring. To our knowledge, this type of valve malfunction has not been previously noted.

Techniques to simplify esophagogastric circular stapled anastomoses.

Stapling devices for end-to-end anastomoses (EEA) have facilitated more rapid and reliable reestablishment of esophagogastric continuity following esophageal resections. Despite their ease of use, various intraoperative problems can arise, especially with the esophageal pursestring or the insertion of the anvil into the fragile, commonly contracted lumen. This paper describes various technical details that are useful adjuncts to allow creation of rapid, consistently successful EEA stapled esophagogastric anastomoses. These techniques are of particular value in the resident teaching setting.

Dissecting intramyocardial hematoma masquerading as a pseudoaneurysm of the left ventricle.

This report presents a case of left ventricular intramyocardial dissection masquerading as a ventricular pseudoaneurysm, as documented by echocardiography, magnetic resonance imaging, and angiography with subsequent confirmation at operation.

Pharmacologic inhibition of scar contracture of intracardiac prosthetic patches.

Significant fibrosis and contracture often cause complications of pericardial and Dacron grafts within the heart. Both continue to be utilized because more suitable materials are unavailable. Newer prosthetic materials and the administration of drugs that might minimize intracardiac fibrosis and contracture after implantation were studied. Measured patches of three prosthetic materials were implanted into the right atrium of 18 dogs: 6 with expanded polytetrafluoroethylene (PTFE or Gore-Tex--W. L. Gore & Associates, Elkton, Md.), 6 with glutaraldehyde-preserved bovine pericardium, and 6 with Dacron patches. The animals were sacrificed after 8 weeks and gross and microscopic pathologic examinations were performed. The patch materials did not intrinsically shrink but all demonstrated marked distortion from the growth of surrounding scar tissue. In a fourth group of eight dogs with intraatrial Dacron patches, four were treated for 8 weeks with oral 3-aminopropionitrile fumarate, and four were not treated. A blinded observed evaluated the adhesions on a scale of 0 = no adhesions or patch distortion, to 4 = dense adhesions with marked distortion of the patches. The untreated dogs had dense adhesions of the lung and pericardium and the patches were encased in scar tissue with marked distortion. The average score was 3.5. The treated dogs had only minimal pleural adhesions and only a thin neointimal covering of the patch with virtually no distortion. The average score was 1.0 (P = 0.0032). The tensile strength of the healed atriotomies was evaluated with stress testing. The intact atrial wall ruptured at a force of 2.60 +/- 0.37 kg/m/sec2, the untreated atriotomy wound at 2.38 +/- 0.18 kg/m/sec2, and the treated atriotomy wound at 2.60 +/- 0.17 kg/m/sec2. There was no statistically significant difference among these groups. No other side effects of the 3-aminopropionitrile fumarate were noted, with only a single superficial wound infection caused by early postoperative trauma. Pharmacologic inhibition of scar formation minimized distortion and contracture of intracardiac prosthetic patches and decreased adhesions. Though further experimental evaluations are necessary, prolonged low-dose oral administration of beta-aminoproprionitrile (BAPN) may be beneficial in preventing complications of prosthetic implants and reoperative cardiac surgery in children.