Absent pulmonary valve associated with tetralogy of Fallot and complete atrioventricular septal defect: report of a case.

A male infant with an extremely rare combination of absent pulmonary valve, tetralogy of Fallot and atrioventricular septal defect presented without symptoms of respiratory distress or congestive heart failure. He underwent successful primary repair at the age of 5 months. The procedure consisted of double-patch repair of the atrioventricular septal defect and right ventricular outflow tract reconstruction with a monocusp transannular patch. Resection or plication of a dilated pulmonary artery was not required. The patient is doing well without any symptoms 5 years after repair.     

Ventricular septal defect with tricuspid pouch with and without transposition. Anatomic and surgical considerations.

In a 10-year review, patients operated on for ventricular septal defect and tricuspid valve pouch were divided into two groups, because the effect of the tricuspid valve pouch is influenced by which ventricle has the higher pressure. Group I comprised patients with ventricular septal defect without transposition of the great arteries and group II, ventricular septal defect with transposition. In 72 of 392 group I patients, the septal tricuspid valve leaflet was incised to expose the edges of the hidden ventricular septal defect to accomplish proper anatomic repair. Forty-eight patients had a tricuspid valve pouch, the diagnosis being established by angiography, echocardiography, or at operation. Ages at operation ranged from 5 months to 22 years and the pulmonary-systemic flow ratio ranged from 1 to 3.4, with 16 being less than 1.5. In one patient the pouch produced a 40 mm Hg pressure gradient in the right ventricular outflow tract. At operation, through a transatrial approach, the tricuspid valve pouch was opened radially, the actual ventricular septal defect patched, and the tricuspid valve leaflet repaired. There were no deaths, no significant intraoperative or postoperative morbidity, and no tricuspid valve dysfunction. The average postoperative hospital stay was 4.8 days. In group II, six of 83 patients operated on for transposition with ventricular septal defect had significant left ventricular outflow tract obstruction from the tricuspid valve pouch. Five of six had a Mustard procedure, two requiring a left ventricular-pulmonary artery conduit, and in two of the six the ventricular septal defect was closed through the pulmonary artery. One patient had heart transplantation after a Mustard repair and tricuspid valve replacement. The sixth patient in group II had a successful arterial switch at 9 years of age, after the presence of left ventricular outflow tract obstruction was proved to be due to the pouch. The presence of a tricuspid valve pouch in group I may lead the surgeon to close false small openings produced by the pouch rather than the actual ventricular septal defect. Incising the pouch is safe and essential for proper exposure and secure closure of the true defect. In group II, the systemic right ventricular pressure can push the pouch into the left ventricular outflow tract, causing significant obstruction, and may contribute to tricuspid valve insufficiency after atrial baffle repair. Arterial switch is preferred because it returns the obstructive tricuspid valve pouch and abnormal tricuspid leaflet to the lower pressure pulmonic right ventricle.     

Creation of an atrial septal defect for acute postperfusion right ventricular failure

Acute postperfusion right ventricular failure following mitral and aortic valve replacement in a patient with severe double-valve incompetence secondary to endocarditis is presented. The situation was reversed by creating an atrial septal defect that decompressed the right ventricle and increased left ventricular filling pressure.     

Pulmonary homograft implantation for ventricular outflow tract reconstruction: early phase results

The pulmonary valve homograft (PH) has been reported to have potential advantages over the aortic valve homograft, including a larger diameter, a thinner wall, and decreased intrinsic calcification. From January 16, 1986, to July 14, 1987, eight consecutive patients underwent repair of congenital cardiac anomalies using a cryopreserved PH. Patients ranged in age from 18 months to 32 years. Diagnoses included tetralogy of Fallot with pulmonary atresia (3 patients); tetralogy with absent pulmonary valve (1 patient); corrected transposition with pulmonic stenosis (1 patient); transposition of the great arteries, ventricular septal defect, and pulmonic stenosis (2 patients); and double-outlet right ventricle with pulmonic stenosis (1 patient). The PH was implanted orthotopically in the patient with absent pulmonary valve, and in the other 7 it was placed as a valved extracardiac conduit. Two of the tetralogy patients with severe bifurcational pulmonary stenosis and another with nonconfluent pulmonary arteries and origin of the left pulmonary artery from a patent ductus arteriosus had their repairs facilitated using the branching pulmonary arterial portion of the PH. There were no hospital or posthospital deaths. Postrepair right ventricular to left ventricular systolic pressure ratios were a mean of 0.35 at 18 hours postoperatively (range, 0.21-0.61). All patients were studied with Doppler and echocardiography after repair. The mean gradient across the PH was 9 mm Hg (range, 2-27 mm Hg), and no pulmonary valve incompetence was present. One patient (12.5%) required reoperation seven months after repair for conduit revision due to compression by the sternum and is now well.(ABSTRACT TRUNCATED AT 250 WORDS)     

Complete atrioventricular canal and tetralogy of Fallot: surgical management

Between 1982 and 1989, nine patients with complete atrioventricular (AV) canal and tetralogy of Fallot underwent successful repair of both anomalies. Seven patients had Down's syndrome. One or more previous palliative shunts had been used in six patients. Associated cardiovascular lesions included persistent left superior vena cava (two patients), patent ductus arteriosus with marked stenosis of the left pulmonary artery (one patient). The diagnosis was confirmed by cardiac catheterization and cineangiography. Closure of the ventricular septal defect was performed through a combined right atrial and ventricular approach in each patient. The right ventricular outflow obstruction was relieved with the use of a transannular patch (4), right ventricular infundibular patch (4) or a valved external conduit (1). There were no hospital deaths. One patient died as a result of meningitis 2 years after repair. Progressive left AV valve incompetence developed in one patient requiring valve replacement. A single atrial approach may not provide the best exposure of the subaortic end of the defect. In this regard, we recommend an additional right ventriculotomy in order to ensure safe closure of the septal defect. Although an adequately shaped isolated patch could be sufficient to close the anterior VSD, we favour a separate patch to minimize the possibility of producing subaortic obstruction. A further method to avoid this subaortic stenosis consists of dividing the common anterior leaflet to the right towards the lateral extent of the infundibular septum.     

Repair of complete atrioventricular septal defect with tetralogy of Fallot

Repair of complete atrioventricular canal with tetralogy of Fallot was performed in 9 patients. Ventricular septal defect was closed through the right atrium using a single polytetrafluoroethylene patch with ample anterior extension to avoid subaortic obstruction. The atrial septal defect was closed with a separate patch. Undivided atrioventricular valve leaflets were sandwiched between the two patches. Right ventricular outflow tract stenosis was relieved by pulmonary valvotomy and an infundibular patch in 7, a supravalvar patch (none transannular) in 6, and right ventricle-to-pulmonary artery conduit in 2. There was one hospital death (1/9, 11%) in a patient with persistent clinically significant postoperative pulmonary stenosis and low cardiac output requiring reoperation and right ventricle-to-pulmonary artery conduit insertion. There was no late mortality. All patients are asymptomatic 0.3 to 5.6 years after operation. Follow-up right ventricular outflow tract gradient ranged from 11 to 43 mm Hg and was 70 mm Hg in 1 patient who later had successful relief of obstruction. Three patients had mitral valve insufficiency; 1 needed reoperation. Aggressive relief of right ventricular outflow tract stenosis with maintenance of pulmonary valve competence and use of two separate patches for closure of the septal defects contribute to optimum immediate and long-term results after repair of this lesion.     

Surgical management of the conal (supracristal) ventricular septal defect

Surgical management of the conal (supracristal) ventricular septal defect differs significantly from the management of the perimembranous (infracristal) ventricular septal defect. The absence of a portion of the conal septum can lead to prolapse of the right cusp of the aortic valve, which predisposes these patients to aortic insufficiency. Between January 1980 and December 1989, 36 children with conal ventricular septal defect underwent intracardiac repair. Diagnosis was by echocardiography, cardiac catheterization, and intraoperative exploration. Preoperative evaluation showed that 26 patients (72%) had aortic valve prolapse and 16 (44%) had aortic insufficiency. Pulmonary-to-systemic flow ratios ranged from 1:1 to 3.5:1 (mean 2.0:1.0). Ten patients (27%) were believed to have clinical congestive heart failure. Age at the time of operation ranged from 2 weeks to 18 years (mean 5.5 years). Operative exposure was through the pulmonary artery (26), aorta (4), right ventricle (3), or right atrium (3). Simultaneous aortic valve suspension for aortic insufficiency was performed in four patients. Operative survival was 100%. Follow-up is complete in all patients and ranges from 0.5 to 9 years (mean 4.3 years). All patients are in normal sinus rhythm. No residual ventricular septal defects have been identified. Twenty-three of 36 patients (64%) have no evidence of aortic insufficiency; 12 of 36 (33%) have trivial or mild aortic insufficiency. One patient with initial severe aortic insufficiency underwent repeat aortic valvuloplasty 3 years after ventricular septal defect closure and aortic valve suspension. No patients have required aortic valve replacement. Surgical management of the conal ventricular septal defect differs from that of the perimembranous ventricular septal defect in two critical aspects. The operative approach should be through the pulmonary artery. This allows the best exposure of the remaining conal septum and the pulmonary and aortic valve leaflets, facilitating closure of the defect without injury to the valves or conduction system. Conal ventricular septal defects should undergo early closure, regardless of shunt volume, to prevent progressive aortic insufficiency.     

Caplike double-horned double-outlet right ventricle. Report of two cases.

Two patients with a rare form of double-chambered, double-outlet right ventricle (DORV) are described. Both patients had associated pulmonary stenosis, ventricular septal defect, and a large tricuspid valve whose chordal attachments crossed the right ventricular outflow tract. The angiocardiographic, echocardiographic, and operative findings were distinctive. The right ventricle was positioned as a "cap" sitting atop the ventricular septal defect at the cephalad end of the left ventricle. The right ventricle, with its two "horns," was likened to a rider straddling a saddle. Tricuspid chordal attachments extended into each of the two right ventricular "horn." No repair was performed in the asymptomatic patient. The other repair involved closure of the tricuspid orifice and insertion of a valved extracardiac conduit from the right atrium to the pulmonary artery.     

Surgical repair of double-chambered right ventricle with or without ventriculotomy

Double-chambered right ventricle is an uncommon congenital anomaly consisting of obstruction of the trabecular zone of the ventricle by an elevated, hypertrophied moderator band, with or without additional muscle bundles(s). During an 18 month period (August 1984 to February 1986) 12 patients underwent repair of double-chambered right ventricle. Their ages ranged from 13 months to 12 years (mean 4.4 years). Associated lesions were ventricular septal defect in 11 patients (three closed spontaneously), atrial septal defect in two, left superior vena cava in three, aortic valve prolapse in one, and mild subaortic stenosis in two. Right ventricular outflow tract gradients were from 30 to 165 mm Hg (mean 77.2 +/- 37.3 mm Hg). The initial six patients (Group 1) were treated in the "classical" manner with a right ventriculotomy for muscle resection and ventricular septal defect closure (4/6). All patients survived. Five of the six required pharmacologic support for signs of mild to moderate right ventricular failure. The right ventricular outflow tract gradient postoperatively was 0 mm Hg in five patients and 25 mm Hg in one patient. Oxygen saturations were checked in the right atrium and pulmonary artery in all six patients, and one patient had a step-up of more than 10%. In the next six patients (Group 2) a previously unreported right atrial-pulmonary artery approach was used to avoid a ventriculotomy. The right ventricular outflow tract gradient was 0 mm Hg in five patients and 5 mm Hg in the sixth. A ventricular septal defect was closed in four of the six patients. There were no deaths, and no patient required inotropic support. Follow-up has been from 2 to 20 months. There have been no late deaths, and all patients are asymptomatic. One patient in Group 1 still has signs of a residual ventricular septal defect at 1 year. No patient has echocardiographic evidence of right ventricular outflow tract obstruction. All patients have remained in sinus rhythm with right bundle branch block. No patient has been recatheterized. Repair of double-chambered right ventricle is easily performed without a ventriculotomy. The atrial exposure of the ventricular septal defect is excellent. The muscle bundle(s) can be visualized easily through an initial transverse pulmonary arteriotomy with completion of resection through the tricuspid valve.     

A new operation for ventricular septal defect with aortic incompetence

Two boys, 10 years of age, with ventricular septal defect and severe aortic insufficiency were treated using a new method. An autograft of anterior pulmonary artery wall and the adjacent leaflet and subpulmonary muscle was excised. The excised pulmonary artery segment was used to replace the prolapsed right coronary cusp, and the autograft muscle was used to close the ventricular septal defect. The right ventricular outflow tract and pulmonary valve were repaired using an autologous pericardial patch with an autologous single cusp. The results in both cases were satisfactory.     

Surgical treatment of double-outlet left ventricle in 2 patients with D-position and L-position of the aorta.

Two cases of double-outlet left ventricle (DOLV) are described, 1 with D-position and 1 with L-position of the aorta. Both patients had situs solitus, atrioventricular concordance with D-relationship of the two ventricles, a ventricular septal defect, and subpulmonary stenosis. The patient with D-position of the aorta was operated on using a technique similar to that for repair of Fallot's tetralogy but including emergency implantation of a bioprosthesis into the pulmonary artery position on the eighth postoperative day. Surgical correction in the patient with L-position of the aorta required the implantation of a composite valved conduit between the right ventricle and the pulmonary artery. The various surgical techniques for correction of DOLV are described, and the literature is reviewed. Postoperative right heart failure in both patients is explained by the impaired function of the right ventricle being exposed to gross pulmonary incompetence or by the persistence of elevated right ventricular pressure.     

De Vega tricuspid annuloplasty for tricuspid regurgitation in children

BACKGROUND: Significant tricuspid valve regurgitation (TR) occurs with other congenital heart defects, typically after repair of right-sided obstructive lesions. Since 1991, we applied the De Vega tricuspid annuloplasty technique for TR in children. METHODS: Forty-one children, aged 5 months to 22.7 years (mean, 9.9 years) underwent 42 De Vega tricuspid annuloplasties for moderate or severe TR during correction of other heart defects. One child had a De Vega during primary ventricular septal defect repair. The remaining patients had prior repair of tetralogy of Fallot or pulmonary atresia, or both (19 patients), double-outlet right ventricle (6 patients), pulmonary stenosis (4 patients), pulmonary atresia and intact ventricular septum (3 patients), complete atrioventricular septal defect (3 patients), and other diagnoses (6 patients). At the time of the De Vega, 37 patients (88%) had pulmonary valve replacement or right ventricular to pulmonary artery conduit replacement. Other procedures included aortic or mitral repair or replacement (6 patients), atrial septal defect and ventricular septal defect closure (5 patients), pulmonary arterioplasty (6 patients), and tracheoplasty (1 patient). RESULTS: There were no deaths at follow-up of 3.4 +/- 2.1 years; 1 child required cardiac transplantation 17 months postoperatively. Early postrepair echocardiography quantified TR as absent or mild (34 patients; 81%), mild-to-moderate (4 patients), moderate (3 patients), and severe (1 patient). The most recent echocardiogram showed moderate TR in 11 patients and severe TR in 2 patients (both with recurrent right ventricular hypertension). One child required tricuspid valve replacement 3 years later and 1 child had redo De Vega at the time of conduit re-replacement. No other child has symptomatic TR, significant tricuspid stenosis, or De Vega-related pacemaker implantation. CONCLUSIONS: The De Vega tricuspid annuloplasty safely provides excellent relief of TR, usually in children undergoing pulmonary valve replacement or conduit replacement. Although echocardiographic TR tends to increase with time (especially with right ventricular hypertension), it rarely requires reintervention or causes symptoms.     

A successful arterial switch operation for the transportation of the great arteries with posterior aorta--with special reference to its anatomical features and possibility of the indication of the Jatene procedure

A five months old infant with the transposition of the great arteries with posterior aorta undergoing arterial switch operation is reported. A pre-operative diagnosis of the transposition of the great arteries with posterior aorta, subarterial ventricular septal defect, anterior pulmonary artery, bilateral conus and fibrous continuity between aortic and mitral valve was made. This diagnosis was confirmed by the open heart surgery. The ventricular septal defect was subaortic from the transected aortic aspect, and the infundibular and trabecular septum was aligned. For these reasons, this subarterial ventricular septal defect was easily closed transaortically. The right coronary artery arose from the left sinus and the left coronary artery from the posterior sinus, so the coronary arterial pattern of this patient was a Shaher type 9. And in addition, another small ostium located in the left sinus gave rise to an additional small branch. Translocation of the coronary arteries was performed. The new pulmonary trunk was reconstructed to the right pulmonary artery so as not to compress the coronary artery and distort the great arteries. This patient was restudied three months after the repair. The right to left ventricular systolic pressure ratio was 0.42 and no pressure gradient was present between the right ventricle and the right pulmonary artery. But a moderate aortic regurgitation was detected. This was caused by deformity of the aortic sinus of valsalva. Only 28 patients with the transposition of the great arteries with posterior aorta were reported in the literature, but there were no reports on the successful surgical treatment for its rare anomaly, to our knowledge.(ABSTRACT TRUNCATED AT 250 WORDS)     

室上嵴上室间隔缺损的外科治疗

本文报道５６例室上嵴上室间隔缺损的外科治疗。男３４例，女２２例，年龄３～３９岁。１９例合并主动脉瓣叶脱垂、主动脉瓣关闭不全或佛氏窦瘤破裂。经右房切口修补室缺２例，右室漏斗切口４７例，肺动脉切９７例。直接修补缺损２６例，补片修补３０例。主动脉瓣成形术１例，主动脉瓣置换术５例。５４例全愈出院，死亡２例。作者认为室上嵴上空间隔缺损应尽早手术，以免并发主动脉瓣损害和佛氏窦瘤破裂；中度主动脉瓣关闭不全可行主动脉瓣成形术，采用肺动脉切口优于右室漏斗部切口。     

Results with the two-patch technique for repair of complete atrioventricular septal defect

From May, 1982, to September, 1983, 9 patients underwent repair of complete AV septal defect. They ranged in age from 11 months to 48 months and in weight from 5.3 kg to 16.5 kg. Seven patients were 24 months old or less. Previous operations included pulmonary artery banding in 1 patient and ligation of a patent ductus arteriosus with repair of coarctation in another. All patients had large left-to-right shunts (mean pulmonary to systemic flow ratio, 3.1), and the 7 young infants had marked pulmonary hypertension. Mitral regurgitation was absent in 2 patients, mild in 3, moderate in 2, and severe in 2. One patient had the right ventricular dominant form of complete AV septal defect. In all instances, repair was done using separate ventricular and atrial patches. Leaflet tissue was not divided, and a trileaflet mitral valve was left in each patient. Eight patients survived operation and are well 3 to 17 months after repair. The single operative death occurred in the patient with right ventricular dominance. Only 1 patient has mild residual heart failure 4 months after operation. Clinically, mitral regurgitation is absent in 4 patients and, at most, mild in the other 4. No patient has a conduction disturbance. Repair of complete AV septal defect is facilitated by using separate patches for the ventricular and atrial components of the defect. Less distortion is created, and a more accurate reconstruction of a competent trileaflet mitral valve can be done.     

全胸腔镜下室间隔缺损修补手术单中心临床分析

目的总结全胸腔镜下室间隔缺损修补手术临床经验。方法回顾性分析2008年5月至2018年12月我院449例行全胸腔镜下心脏手术的室间隔缺损患者的临床资料,男232例、女217例,年龄3~55(17.3±11.2)岁。结果449例患者手术时间为1.8~4.0(2.4±0.3)h,体外循环时间42~136(64.2±11.6)min,升主动脉阻断时间17~84(28.4±10.7)min,术后呼吸机辅助时间3~45(6.9±3.8)h,术后住重症监护室时间16~68(20.5±5.6)h,术后胸腔闭式引流量50~1060(213.1±117.2)mL,术后住院时间5~16(6.9±1.3)d。全组患者均治愈出院,无中转正中开胸患者。出现并发症11例(2.4%),包括术中再次手术1例,术后胸腔镜下二次止血3例,切口感染3例,室间隔缺损残余漏2例,股动脉切口狭窄合并血栓栓塞1例,右侧气胸1例。患者术后随访1~128(72.2±33.9)个月,无再次手术患者,室间隔缺损残余漏2例,二尖瓣及主动脉瓣轻中度关闭不全各1例,心功能明显好转。结论全胸腔镜下室间隔缺损修补手术具有并发症少、术后恢复快及安全可靠等优势,近中期疗效良好。     

Transatrial-Transpulmonary Repair of Atrioventricular Septal Defect with Right Ventricular Outflow Tract Obstruction

A bstract Twenty patients had a repair of an atrioventricular septal defect with tetralogy of Fallot (n = 13) or double outlet right ventricle (n = 7). Mean age was 3.5 years. Surgical technique included transatrial-transpulmonary resection of right ventricular outflow tract obstruction and transatrial two patch repair of the atrioventricular septal defect. Ten patients required a transannular patch and one patient had a right ventricle-pulmonary artery conduit placed. There was no hospital mortality, and mean hospital stay was 15 days. One patient had late sudden death of unknown cause. Six patients have required reoperation because of residual ventricular septal defect (VSD), mitral incompetence, residual right ventricular outflow tract obstruction, and/or conduit stenosis. No patient was reoperated on because of left ventricular outflow tract obstruction. Fifteen patients are asymptomatic, one has exertional dyspnea, and two have intermittent occasional bronchospasm. The transatrial-transpulmonary two patch repair and extensive relief of right ventricular outflow tract obstruction have given good immediate results. Reoperation rate has been high mainly due to residual VSD and mitral incompetence. ( J Card Surg 1993; 8:622–627 )     

A case report of total repair in a 71-year-old patient with tetralogy of Fallot

A 71-year-old male is presented as ever the oldest patient of tetralogy of Fallot who underwent successful radical surgery. Heart murmur was pointed out at the age of 10 years. The patient consulted us because of dyspnea and cough, and was noted to have cyanosis and clubbing fingers. Polycythemia was also detected by hemoglobin of 20.8 g/dl and hematocrit of 58.4%, and a low PaO2 of 48.5 mmHg at room temperature was pointed out. Preoperative echocardiography and cardiac catheterization indicated a ventricular septal defect, overriding of the aorta, and right ventricular outflow tract stenosis with a pressure gradient of 115 mmHg between the right ventricle and the main pulmonary artery. Under cardiopulmonary bypass, the ventricular septal defect was closed with a dacron patch and the right ventricular outflow tract was enlarged by a patch of collagen-coated vascular graft with a commissurotomy of the pulmonary valve. Postoperatively, cyanosis disappeared and the pressure gradient was decreased to 26 mmHg.     

Aneurysm of pericardial right ventricular outflow tract patches.

Among 1,022 patients who underwent repair of tetralogy of Fallot, 252 received a pericardial patch of the right ventricular outflow tract; of these, 10 subsequently developed an aneurysm of the right ventricular outflow tract. Cardiac catheterization and angiography revealed moderate pulmonary insufficiency in all patients, a residual pressure gradient in the right ventricular outflow tract in 7, and a residual ventricular septal defect in 2 patients. Reoperation was indicated in 8 patients because of progressive distention of the aneurysm, residual infundibular or pulmonary artery stenosis, and recurrent ventricular septal defect. Reconstruction of the right ventricular outflow tract was accomplished by resection of the aneurysm and insertion of a woven Dacron patch in 5 patients, primary suture of the pulmonary artery in 2, and implantation of a woven Dacron conduit containing a Björk-Shiley cardiac valve prosthesis in 1 patient. There were no early or late deaths. When reconstruction of the right ventricular outflow tract is necessary, we recommend a woven Dacron patch because pericardium may form an aneurysm.     

Autologous Pericardium Patch Aneurysm after Ventricular Septal Defect Closure and Arterial Switch Operation

Abstract   A neonate with L-transposition of the great arteries with ventricular septal defect underwent complete repair using fresh autologous pericardium to close the ventricular septal defect as well as to reconstruct the neo pulmonary artery sinuses. Four months later, the child came back with right ventricular inflow obstruction related to aneurysmal pericardial patch, severe tricuspid regurgitation, and severe supra-valvular pulmonic stenosis. At reoperation, there was a redundant, aneurysmal pericardial patch densely adherent to the septal and posterior leaflets of the tricuspid valve, which was damaged. The pericardial patch was replaced, the pulmonary artery enlarged, and tricuspid valve repaired. Postoperative course was uneventful, but residual moderate tricuspid regurgitation required intensive medical treatment.