Extended end-to-end repair and enlargement of the entire arch in complex coarctation.

BACKGROUND: Treatment of hypoplasia of the entire arch in coarctation is a surgical challenge. The current approaches have technical difficulties, high recurrence rates, and increased morbidity and mortality. METHODS: Over a 14-month period, a combined extended end-to-end repair with patch enlargement of the concavity of the entire arch was performed in 6 neonates and 1 infant. Through a midsternotomy and using cardiopulmonary bypass and hypothermia, extended end-to-end repair was performed initially leaving the proximal anastomosis open. The enlarging polytetrafluoroethylene patch was then sutured starting at the incised descending aorta distal to the extended end-to-end repair and continued retrogradely through the transverse arch to the ascending aorta proximal to the aortic cannulation site. One neonate had a patent ductus arteriosus and another had ventricular septal defect closure. One neonate had arterial switch and 3 had Norwood-type procedures performed with the enlarging patch extended to the pulmonary artery anastomosis. The remaining infant had arch enlargement performed after an arterial switch procedure and extended end-to-end repair. RESULTS: All patients did well and showed no residual gradient up to 1 year follow-up. Two patients successfully had bidirectional Glenn shunt at 9 months of age, and one had closure of residual arterial septal defect at 8 months of age. CONCLUSION: The combined extended end-to-end repair and arch enlargement procedure should minimize recurrence rates because of a tension-free enlargement of the entire aortic arch and elimination of the coarctation ridge and ductile tissues. Combined with the arterial switch and Norwood-type procedures, the approach results in a large neoaorta.

Semilunar valve switch procedure: autotransplantation of the native aortic valve to the pulmonary position in the Ross procedure.

BACKGROUND: The Ross procedure has gained wide acceptance in young patients with aortic valve disease. The durability of the pulmonary autograft in the aortic position has been proved, with up to 24 years of follow-up. The homograft pulmonary valve, however, has limited longevity. To circumvent this problem we harvested, repaired, and reimplanted the native aortic valve with intact commissures in the pulmonary position in 13 patients undergoing the Ross procedure for aortic insufficiency. METHODS: The cause of aortic insufficiency was rheumatic in 6 patients, congenital in 4, post-aortic valvotomy in 2, and bacterial endocarditis in 1. Patient age ranged from 5 to 45 years (mean, 17+/-9 years). Root replacement technique with coronary artery reimplantation was used. In the first 4 patients, the native aortic valve was sutured into the right ventricular outflow tract, and a polytetrafluorethylene patch was used to reconstruct the main pulmonary artery. In the last 9 patients, the aortic valve and polytetrafluorethylene patch were made into a conduit by another surgeon while the left-sided reconstruction was performed. RESULTS: All patients had marked reduction of left ventricular dilation and good function of the reimplanted native aortic valve, with up to 50 months of follow-up (mean, 29.9+/-14.2 months; range, 12 to 50 months). Two patients died 15 and 26 days, respectively, of a false aneurysm rupture at the distal aortic anastomosis. In the remaining 11 patients, 9 (82%) had mild or absent, and 2 (18%) had mild to moderate, neoaortic valve regurgitation. Similarly, 9 patients (82%) had mild or absent, and 2 (18%) had mild to moderate, neopulmonary valve regurgitation. Mild neopulmonary valve stenosis was present in 6 patients (54%) (mean gradient, 29+/-4 mm Hg; range, 25 to 35 mm Hg). All surviving patients are in functional New York Heart Association functional class I. CONCLUSIONS: We conclude that use of the native aortic valve with the Ross procedure makes the procedure attractive and potentially curative. The diseased aortic valve works well in the pulmonary position because of lower pressure and resistance. The valve leaflets should remain viable and grow in both the pulmonary and aortic positions because they derive nutrition directly from the blood.

The pericardial membrane pulmonary monocusp: surgical technique and early results.

Long-term pulmonary insufficiency resulting from simple transannular patching of the right ventricle outflow tract will ultimately lead to deterioration in right ventricular function. Previously, monocusps constructed from xenografts, homografts, fascia lata, and autologous pulmonary artery wall have been utilized to minimize pulmonary regurgitation and its deleterious effect on right ventricular function. However, these tend to degenerate in the long term, necessitating reoperation. To circumvent this problem we have used a monocusp constructed from 0.1-mm polytetrafluorethylene (PTFE, pericardial membrane) clinically demonstrated to be resistant to issue ingrowth and degeneration. Seven children (5 tetralogy of Fallot, 2 pulmonary stenosis) who required division of a small pulmonary annulus underwent monocusp construction with 0.1-mm PTFE. Three patients had previous corrective surgery. One of these patients had a bovine pericardial monocusp placed 8 years previously, which degenerated. Of the remaining two patients, one had a pulmonary valvotomy as a neonate, the other repair of tetralogy of Fallot with a transannular patch. At a mean (+/- standard deviation) follow up to 17+/-5.8 months all patients are alive and are New York Heart Association (NYHA) Class I. Echocardiography demonstrates mild pulmonary insufficiency (PI) in 2 patients, mild to moderate PI in 4, and moderate to severe PI in 1. The presence of a pericardial membrane monocusp in the pulmonary position may, in the long term, prevent the deleterious effects of transannular patching on right ventricular dysfunction and be more resistant to degenerative changes characteristic of monocusps constructed of native pericardium or allogeneic tissue.

Modified Konno-Rastan procedure for subaortic stenosis: indications, operative techniques, and results.

BACKGROUND: Diffuse or unresectable subaortic stenosis (SAS) necessitates an aggressive surgical approach for the elimination of left ventricular outflow tract obstruction. In this article we report our experience with the modified Konno-Rastan procedure, with inherent preservation of the native aortic valve and annulus, in the treatment of diffuse or unresectable SAS. METHODS: Sixteen children (age range, 21 months to 18 years) underwent the modified Konno-Rastan procedure through either a transventricular (n = 12) or a transatrial approach (n = 4) to the conal septum. Indications for operation were recurrent SAS (n = 3), hypertrophic obstructive cardiomyopathy (n = 3), tunnel stenosis (n = 2), SAS related to a canal (n = 3), and SAS after ventricular septal defect closure (n = 5). Eleven patients had undergone previous procedures and 5 underwent the modified Konno-Rastan procedure as their primary operation. RESULTS: The mean preoperative left ventricular outflow tract gradient of 50 +/- 17 mm Hg was reduced to 3 +/- 7 mm Hg (p < 0.001) after surgical repair. Postoperative complications included sternal infection (n = 1), heart block (n = 2), mediastinal bleeding (n = 1), and renal and cerebral ischemia (n = 1). There was 1 late postoperative death caused by pneumonia 2 years after operation (6.2% mortality rate). The mean follow-up period was 62 +/- 39 months and all patients had complete relief of preoperative symptoms and were in New York Heart Association class I. One patient underwent a successful redo modified Konno-Rastan procedure 7 years after the first operation for residual left ventricular outflow tract obstruction immediately below the aortic valve. One patient is awaiting reoperation for aortic incompetence unrelated to conal enlargement 1.5 years after the first procedure. CONCLUSIONS: The modified Konno-Rastan procedure represents an excellent therapy for diffuse or unresectable SAS in patients with a normal aortic valve. In addition, it produces excellent results in a limited number of patients with hypertrophic obstructive cardiomyopathy, in whom the Morrow procedure traditionally has been performed. Although it usually is performed through a transventricular approach, the modified Konno-Rastan procedure also can be performed through a transatrial approach; this is particularly useful in patients who have had previous ventricular septal defect closure associated with SAS occurring proximal to the prosthetic patch.

Cost-effectiveness of transaxillary muscle-sparing same-day operative closure of patent ductus arteriosus.

Transaxillary muscle-sparing patent ductus arteriosus closure performed as same-day surgery is described in 10 patients. This approach provides a superb cosmetic result while obviating the need for thoracostomy tube placement.

Fenestrated fontan procedure: evolution of technique and occurrence of paradoxical embolism.

The Fenestrated Fontan procedure (FFP) has improved outcome in high risk patients. The technique is evolving, however, and complications are not fully known. Over a 3-year period 13 patients (mean age 35 +/- 29 months) underwent an FFP in our institution. In the first two patients the fenestration had to be created because of high right atrial pressure and low cardiac output; in 11 patients the FFP was planned. In three patients the sutures for the adjustable fenestration were crossing the defect. In 10 patients, purse-string sutures were placed around but not across the defect. Because large fenestrations were created in 11 patients (8-12 mm) Glenn shunts were performed to improve arterial saturation. The postoperative course was relatively uneventful, with chest tubes being removed 1-8 days (mean 4 +/- 3 days) postoperatively and the hospital stay ranging from 7 to 27 days (mean 14 +/- 6 days). One patient had bleeding and another had a mediastinal abscess. The first patient died (7.6%) because of hemodynamic instability due to prolonged cardiopulmonary bypass from the creation and enlargement of the fenestration. One patient had a paradoxical cerebral embolism from clots that formed on the sutures crossing the fenestration. Because of this problem the remaining patients were placed on salicylates while awaiting closure of their fenestration. All 12 patients had their fenestrations closed, performed under local anesthesia in 9, at mediastinal abscess drainage in 1, and spontaneously in 2. We conclude that creation of large fenestrations in combination with Glenn shunts and the use of adjustable fenestrations are viable modifications of the FFP. The use of purse-string sutures around the fenestration and antiplatelet drugs can probably minimize the occurrence of paradoxical embolism.

Management of arch hypoplasia after successful coarctation repair.

BACKGROUND: Pronounced arch obstruction can be seen after a well-repaired coarctation, and this probably results from the failure of a somewhat hypoplastic arch to grow or from clamp injury at the time of the initial repair, or from both causes. Because of mediastinal adhesions and minimal collateral circulation, use of extraanatomic bypass grafts appears to be the preferred approach. METHODS: Six children or young adults presented with arch obstruction over a 3-year period. Their mean age was 13.5 +/- 4 years, and the mean interval from the time of the initial repair was 10 +/- 4 years. The mean age of the patients at the time of the initial repair was 3.2 +/- 5 years. Symptoms included exertional headache and chest pain. The mean systolic gradients, as shown by echocardiography and cardiac catheterization, were 34 +/- 7 mm Hg and 33 +/- 6 mm Hg, respectively. Repair was accomplished through a midsternotomy using a polytetrafluoroethylene patch placed in the concavity of the arch, which extended from the ascending to the descending aorta. Dissection was kept close to the aorta and arch to minimize injury to the phrenic and recurrent laryngeal nerves. Cardiopulmonary bypass and moderate hypothermia (25 degrees to 27 degrees C bladder temperature) without circulatory arrest were used. RESULTS: All patients were discharged home 4 to 20 days postoperatively (mean, 7 +/- 6 days). All patients were found to be normotensive at a mean follow-up of 1.3 +/- 1 years. Postoperative echocardiograms, which were obtained in all patients, revealed no residual gradients. Exercise blood pressure was evaluated in 2 patients and found to be normal. CONCLUSIONS: Transsternal arch enlargement using cardiopulmonary bypass and moderate hypothermia without circulatory arrest is an attractive and safe approach for the treatment of arch obstruction after coarctation repair. Unlike the use of extraanatomic bypass grafts, it allows complete relief of the obstruction, unhampered aortic growth, the minimal use of foreign material, and a repair that is protected deep within the mediastinal space.

Recognition and management of obstructed pulmonary veins draining to the coronary sinus.

BACKGROUND: Obstruction of the pulmonary veins in total anomalous pulmonary venous drainage to the coronary sinus is generally considered rare. However, if it is present, the usual treatment of unroofing the coronary sinus will lead to a poor result. METHODS: Four patients with total anomalous pulmonary venous drainage to the coronary sinus with obstruction were identified over a 14-month period. Three patients in whom the diagnosis of obstruction was not made underwent coronary sinus unroofing. Retrospective review of the preoperative echocardiograms and Doppler studies showed the presence of obstruction in the vertical vein in 2 patients and in the branches in the other. In the fourth patient, obstruction in the vertical vein was recognized preoperatively with echocardiography and Doppler study. This patient underwent direct common pulmonary vein-left atrial anastomosis. RESULTS: All 3 patients who had coronary unroofing were seen with obstructed pulmonary veins 2 to 7 months postoperatively. After reoperation, 1 died, and the other 2 have done relatively well 3 1/2 and 15 months postoperatively. The patient who had an anastomosis between the common pulmonary vein and the left atrium is doing well 18 months postoperatively. CONCLUSIONS: Obstruction in total anomalous pulmonary venous drainage to the coronary sinus is not as rare as previously reported. To improve outcome, its presence should be sought using complete echocardiography including Doppler studies. When obstruction is present, transection of the vertical vein and common pulmonary vein-left atrial anastomosis through the superior approach is an attractive technique that also eliminates the right-to-left shunting associated with coronary sinus unroofing and simplifies closure of the atrial septal defect.

Clinical improvement after revision in Fontan patients.

BACKGROUND: Arrhythmias, decreased exercise tolerance, or malabsorption will develop in a significant number of Fontan patients. Fontan revision consisting of creation of lateral atrial tunnel, reconnection of the Glenn shunt when present, or both appears to improve these patients. METHODS: Over a 34-month period, 9 patients underwent Fontan revision. The mean age was 11 +/- 5 years and the mean interval from Fontan operation to revision was 3 +/- 2 years. The reason for revision included marked impairment in exercise capacity, inability to go to school consistently, and chronic fatigue in 6 patients, 3 of whom also had serious atrial arrhythmias. Five of the 6 patients had a classic Glenn shunt. The mean right atrial pressure was greater than the pressure of the Glenn shunt (20 +/- 1.6 versus 17 +/- 0.8 mm Hg). Three of the 6 patients also showed a significant gradient between the right or left pulmonary artery wedge and ventricular end-diastolic pressure, indicating pulmonary vein obstruction from the bulging atrial septum or partitioning patch (13 +/- 3 versus 6.8 +/- 1 mm Hg). The remaining 3 patients had revision because of malabsorption (1), hepatomegaly and obstructed right pulmonary veins from bulging atrial septum (1), and tricuspid insufficiency (1). Fontan revision was accomplished with creation of a lateral atrial tunnel and Glenn reconnection in 6 patients, Glenn reconnection in 2, and creation of a lateral atrial tunnel in 1. Four patients had additional procedures. RESULTS: One patient died of Pseudomonas pneumonia. Early extubation, chest tube removal, and postoperative hospital discharge were accomplished in 8 patients (mean = 1.4 +/- 1, 2.8 +/- 1, and 8 +/- 3 days, respectively). One patient died 8 months postoperatively of brain damage after ventricular fibrillation from attempted cardioversion for atrial flutter. The remaining patients had marked improvement in exercise capacity with ability to consistently go to school, improvement in duration and tolerance to arrhythmias on less medication, and resolution of malabsorption up to 37 months postoperatively (mean, 20 +/- 12 months). CONCLUSIONS: We conclude that creation of lateral atrial tunnel with excision of a bulging atrial septum or atrial partitioning patch that causes pulmonary venous obstruction, reconnection of the Glenn shunt, which allows better distribution of flow based on the pulmonary vascular bed and resistance of each lung, or a combination of these procedures will improve Fontan patients.

Early pulmonary homograft failure from dilatation due to distal pulmonary artery stenosis.

Early progressive pulmonary homograft insufficiency developed in an 11-month-old infant after repair of truncus arteriosus because of dilatation secondary to the presence of residual distal pulmonary artery stenosis and hypoplasia. Before repair, the pulmonary artery branches were discontinuous, with the right pulmonary artery being somewhat hypoplastic and originating from the trunk, and the left pulmonary artery supplied by a modified Blalock-Taussig shunt created in the newborn period. At repair, a pulmonary homograft was used to connect the branches. Progressive cardiomegaly and oxygen dependance occurred 3 weeks postoperatively. Cardiac catheterization showed systemic right ventricular pressure, severe homograft insufficiency, and residual distal pulmonary artery stenosis and hypoplasia. On reoperation at 3 months postoperatively, the homograft annulus diameter increased from 14 mm to 16 mm. Dilatation and insufficiency probably occurred because the right ventricle and homograft distal to the obstruction functioned as a unit during systole. The problem might have been minimized with the use of aortic homograft, which is thicker, or annular reinforcement with a synthetic material.

Regression of hypertrophic cardiomyopathy after modified Konno procedure.

BACKGROUND: Septal myotomy-myectomy has been known to decrease the incidence of sudden death and produce regression in hypertrophic obstructive cardiomyopathy. Use of beta-blockers or calcium-channel blockers generally does not cause regression of the disease. METHODS: Having successfully performed modified Konno procedures in 13 patients with effective relief of diffuse subaortic stenosis, we applied the procedure in 2 patients with hypertrophic obstructive cardiomyopathy. Both patients (18 and 12 years old, respectively) presented with syncope, angina at rest, and dyspnea despite being on calcium channel blocker therapy. The echocardiographic outflow gradients were 66 mm Hg and 88 mm Hg, respectively, with moderate mitral regurgitation. RESULTS: Both patients had uneventful postoperative course. At 2 years and 1.5 years postoperatively, both patients were free of angina and syncopal episodes. Echocardiography showed absence of outflow gradients and mitral regurgitation. In 1 patient the septal and posterior wall thickness decreased from 3.4 and 1.7 cm preoperatively to 2.6 and 0.9 cm, respectively, postoperatively. In the other patient, the thickness decreased from 2.4 and 0.9 cm preoperatively to 0.8 and 0.7 cm, respectively, postoperatively. Left atrial diameter decreased from 5.4 to 4.7 cm in 1 patient, 3.5 to 2.6 cm in the other. CONCLUSIONS: We believe that the modified Konno procedure could produce more effective relief of obstruction and, therefore, significant regression and further reduction in sudden death in hypertrophic obstructive cardiomyopathy. On the basis of our experience, albeit limited, we encourage its application.

Alternatives in biventricular repair of double-outlet left ventricle.

Wide variation in morphology of double-outlet left ventricle allows numerous surgical alternatives that require sorting out to develop a more organized approach. There is a high association between tricuspid abnormalities and right ventricular hypoplasia with double-outlet left ventricle that calls for either Fontan-type procedure or biventricular repair. With pulmonic stenosis, biventricular repair has been accomplished using right-sided conduits. When pulmonic stenosis is mild or absent, repair techniques without conduits depend on the commitment of the ventricular septal defect (VSD). With subaortic VSD and mild pulmonic valvar stenosis, we successfully performed translocation of the main pulmonary artery and valve to the right ventricle on 2 patients (ages 32 and 8 months). Both patients are doing well 2 years and 1 year postoperatively. Others have successfully connected the right ventricle to the pulmonary artery with intraventricular baffle by enlarging a subaortic VSD or when the VSD is either subpulmonic or doubly committed. With subaortic VSD, although it has not been reported, biventricular repair can also be accomplished using a right ventricle-to-aorta baffle combined by either atrial or arterial switch. We believe that a simplified management plan can be formed in double outlet left ventricle based on the size of the right ventricle, presence of pulmonic stenosis, and commitment of the VSD. Whenever possible, translocation of the main pulmonary artery and valve or intraventricular repair should be accomplished in double-outlet right ventricle minimizing the use of right-sided conduits and reoperation.

Safety of patent ductus arteriosus closure in premature infants without tube thoracostomy.

During a 30-month period, 34 premature infants underwent surgical closure of a patent ductus arteriosus. The mean gestational age at birth was 25 +/- 0.3 weeks and the mean age at the time of operation was 3 +/- 0.3 weeks (mean weight, 829 +/- 54 g). Indomethacin therapy had failed in 32 patients, and 2 had contraindications to its use. The initial 8 patients had parascapular incision and ligation of the patent ductus arteriosus; the last 26 patients had a short transaxillary incision and clipping. The average duration of the operation from the time of incision to skin closure was 36 +/- 2 minutes (range, 15 to 65 minutes). One patient (3%) needed chest tube insertion intraoperatively because of visceral pleura disruption. Two patients (5.8%) had a "small pneumothorax" (< 10% of the lung field) that resolved within 24 hours. There was no morbidity or mortality directly related to the operative procedure, although 3 patients (8.8%) ultimately died from problems related to their severe prematurity. We conclude that surgical closure of patent ductus arteriosus without chest tube drainage can be accomplished safely in premature infants. Postoperative nursing care is simplified and the cost is reduced because the need for the chest tube and drainage system is eliminated and the number of chest radiograms needed postoperatively is reduced.

Semilunar valve switch in aortic insufficiency.

The use of the Ross procedure in young patients is gaining wider acceptance. The need for a foreign pulmonary valve that will require replacement, however, is a serious drawback. To circumvent this problem, we reimplanted the native aortic valve in the pulmonary position in four patients (ages 12, 15, 15 and 17 years old) operated on utilizing the Ross procedure for aortic insufficiency. One patient had congenital and three isolated rheumatic aortic insufficiency. The root replacement technique with coronary artery reimplantation was used. All patients did well initially with marked reduction of left ventricular dilatation and good function of the reimplanted native aortic valve. One patient, however, died a month later from rupture of a false aneurysm that developed at the pulmonary autograft to ascending aorta anastomosis. We feel that the use of the native aortic valve in the pulmonary position makes the Ross procedure more attractive and potentially curative. The diseased aortic valve works well in the pulmonary position because of lower pulmonary artery pressure and resistance.

Discrete subvalvular aortic stenosis in the Beckwith-Wiedemann syndrome.

Various congenital cardiac malformations have been described in patients with Beckwith-Wiedemann (BW) syndrome, including reversible obstructive subaortic stenosis in one patient. We herein present a case of a 2.5-year-old black boy with BW syndrome and discrete subvalvular aortic stenosis of the membraneous type. Such association of these two entities has previously not been documented.

The use and diagnostic significance of thallium-201 SPECT imaging in a case of anomalous left coronary artery.

A 6-year-old female with an anomalous left coronary artery (ALCA) originating from the main pulmonary artery underwent a resting Tl-201 SPECT examination before and after surgical reimplantation. Preoperative Tl-201 SPECT images demonstrated decreased perfusion to the anterior wall and a dilated left ventricle. Post operative Tl-201 SPECT images showed marked improvement of perfusion to the anterior wall as well as a decreased left ventricular size. This is one of the few documented demonstrations of a perfusion defect due to an ALCA in a young child using Tl-201 SPECT imaging methods in conjunction with other tests.

Echocardiographic evidence for impaired myocardial performance in children with type I diabetes mellitus.

Thirty-three children with type I diabetes mellitus and 51 normal children underwent M-mode echocardiography. Abnormalities of myocardial performance were present in many of the diabetic children. The mean end-systolic volume of the left ventricle was greater in diabetics compared to control subjects. Mean ejection fraction, minor axis shortening, and velocity of circumferential fiber shortening were decreased in the diabetics. There was no evidence of increased myocardial mass in these diabetic children. There was no correlation between myocardial dysfunction, clinical assessment of control, or glycohemoglobin in the diabetic children.