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Biomedical subjects

S Y DeLeon

Publications and source records attributed to S Y DeLeon.

At least 19 recordsLinked to original sources

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.

Anastomosis, Surgical↗

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.

Adolescent↗

Intraatrial mitral valve insertion with native valve preservation in children.

Two patients underwent intraatrial mitral valve insertion for an unsuccessful valvotomy for severe mitral stenosis and left-sided atrioventricular valve insufficiency associated with corrected transposition utilizing a porcine valve from a valved conduit with preservation of the native valve. The valves were inserted using continuous suture distally at the mitral annulus and proximally at the pulled atrial wall distal to the pulmonary veins. Both patients had uneventful hospital course and are doing well at up to 6 months postoperatively. This approach provides a viable option for congenital mitral stenosis or insufficiency in children.

Bioprosthesis↗

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.

Adolescent↗

Anomalous right coronary artery from the pulmonary artery in Taussig-Bing anomaly.

The presence of associated anomalies in patients with double-outlet right ventricle can significantly alter surgical intervention. Preoperative delineation of these anomalies can facilitate surgical planning and improve outcome. We describe a case in which the right coronary artery and anterior descending coronary artery arose from the pulmonary artery in a patient with double-outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly). Recognition of this important anomaly prevented significant intraoperative myocardial damage by altering techniques of cardioplegia administration for myocardial preservation.

Abnormalities, Multiple↗

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.

Coronary Vessel Anomalies↗

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.

Adolescent↗

Simultaneous repair of pectus excavatum and congenital heart disease.

Most repair of heart lesions and pectus deformity has been performed in adult patients using long incisions, sternal splits, excision of deformed cartilages, and sternal turnover operations that could result in poor cosmesis and chest growth in children because of sternal devascularization. We performed simultaneous pectus repair and atrial septal defect closure in 2 children using a short longitudinal incision and avoiding a transverse or longitudinal sternal split. After extraperichondrial excision of the deformed cartilages and mobilization of the sternum from the neurovascular bundles, a transverse wedge of sternum was removed at the level of the third cartilages, allowing cephalad retraction of the sternum and providing excellent exposure for the intracardiac operation. The cosmetic appearance remains excellent in both patients at 1 and 4 years postoperatively.

Child, Preschool↗

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.

Constriction, Pathologic↗

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.

Activities of Daily Living↗

Use of the native aortic valve as the pulmonary valve in the Ross procedure.

The placement of a foreign valve in the pulmonary position using the Ross procedure requires reoperation. To circumvent this problem, we devised a method of reimplanting the native aortic valve in the pulmonary position, and successfully performed this procedure in a 12-year-old diabetic boy operated on for the treatment of aortic insufficiency. Although diseased, the reimplanted aortic valve functioned well, with trivial stenosis and insufficiency. This modification offers patients with aortic valve disease a potentially curative operation.

Aortic Valve↗

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.

Blood Loss, Surgical↗

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.

Adolescent↗

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.

Cardiac Surgical Procedures↗

Recognition and management of accessory mitral tissue causing severe subaortic stenosis.

Failure to recognize the presence of accessory mitral tissue causing subaortic stenosis can lead to not only the performance of inappropriate operations, but the persistence and recurrence of obstruction or even death. Over a 12-month period, we treated 2 children with severe subaortic stenosis caused by accessory mitral tissue. In 1 patient, who was 4 years old, the echocardiogram showed the accessory mitral tissue to be attached to the anterior mitral leaflet and ballooning into the subaortic area. The other patient, as a newborn, underwent simultaneous repair of a complete canal defect and coarctation. Two years later, the patient was seen because of syncopal episodes, progressive mitral insufficiency, and subaortic stenosis thought to be caused by anterior displacement of the anterior mitral leaflet. Mitral valvuloplasty and a conal enlargement procedure were planned. Intraoperatively, after the mitral valvuloplasty had been done, the subaortic stenosis was found to be due to a tight subaortic ring formed by accessory mitral tissue located at the septum and its fibrous extension to the anterior mitral leaflet. In both patients, excision of the accessory mitral and fibrous tissues resulted in a wide-open subaortic area. Both patients had an uneventful hospital course, and follow-up echocardiography showed no noteworthy residual left ventricular outflow gradient. We believe that increased awareness and sophisticated echocardiographic techniques should lead to an increased recognition of accessory mitral tissue causing subaortic stenosis. Simple resection of the accessory mitral tissue and its secondary fibrous tissues can be curative.

Angiography↗

Transsternal repair of coarctation and associated cardiac defects.

Over a 13-year period, 20 infants and children underwent transsternal approach for repair of coarctation and associated cardiac defects. Fifteen patients (75%) were operated on in the last 6 years. Thirteen patients (group 1) had intracardiac shunts and 7 (group 2), intracardiac obstruction or valvular insufficiency. Group 1 had a mean age of 0.8 +/- 1.9 years versus 4 +/- 3 years for group 2 (p = 0.05). There were 12 patients (92%), 7 months old or less in group 1. Aortic arch hypoplasia was present in 6 patients in group 1. A large patent ductus arteriosus was present in 5 of these 6 patients versus no patent ductus arteriosus in patients without aortic arch hypoplasia (p = 0.006). The mean pulmonary blood flow to systemic blood flow ratio in group 1 was 3.8 +/- 2 and the mean right ventricular to left ventricular ratio, 0.8 +/- 0.2. The coarctation repair fell mostly into three types: side patch aortoplasty (8), ductal tissue excision and patch aortoplasty of the concavity of the aortic arch (6), and subclavian aortoplasty (4). There was one early death (5%) which was due to sepsis in a newborn. Another newborn who had subclavian aortoplasty needed a left carotid artery--descending aorta bypass conduit early because of aortic arch hypoplasia. All patients were followed to 12 years (mean follow-up, 4.3 +/- 3.5 years). There were no late deaths. Two patients had recurrent coarctation, 1 after an end-to-end repair and the other because of incomplete arch enlargement after a side patch aortoplasty.(ABSTRACT TRUNCATED AT 250 WORDS)

Aorta, Thoracic↗

Disruption of sutured pulmonary valve in Fontan operation.

Two patients who had undergone a Fontan operation presented late with considerable disruption of a sutured pulmonary valve. Both patients had increasing ascites, decreased exercise tolerance, atrial arrhythmias, high right atrial pressure, and a large ratio of pulmonary blood flow to systemic blood flow. At operation, the main pulmonary artery was closed either by suturing the anterior and posterior walls together immediately distal to the pulmonary valve or by reinforcing the resutured pulmonary valve with a polytetrafluoroethylene patch. Both patients had an uneventful postoperative course, with disappearance of the symptoms and return of sinus rhythm. Although it is tempting to simply suture the usually thickened pulmonary valve in the Fontan operation, approximation of the pulmonary artery walls or patch reinforcement is necessary to minimize disruption.

Adult↗