Complete Surgical Repair Research Articles

Rupture of Aneurysmal Circumflex Coronary Artery Into the Left Atrium After Ligation of Its Arteriovenous Fistula

Aneurysmal circumflex coronary artery (Cx) with fistulous connection to the coronary sinus is a rare clinical entity that usually remains asymptomatic until later in life, so the ideal therapeutic strategy is poorly defined. The timing of surgical treatment for asymptomatic patients is a big issue, and whether to leave or exclude the diffuse aneurysm in addition to ligation of the fistula is controversial, considering the native myocardial circulation. Complete surgical repair, including exclusion of a diffusely aneurysmal Cx and coronary revascularization to a graftable branch in the circumflex area combined with ligation of its fistula, is quite challenging and sometimes fatal because of a broad posterolateral myocardial infarction without revascularization caused by a lack of graftable branches. A case of diffuse aneurysmal Cx, which ruptured into the left atrium after surgical ligation of its fistulous connection to the coronary sinus, is presented. Simple ligation of the fistula, leaving a gigantic aneurysmal circumflex artery, is hazardous for later rupture and should be avoided. Therapeutic strategies for this complex disorder are discussed, including the optimal timing of surgical treatment.

Successful use of a new Amplatzer® Vascular plug for percutaneous closure of a large aortopulmonary collateral artery in a pulmonary atresia with ventricular septal defect prior to complete repair

We report the case of a 14 year-old girl with a pulmonary atresia with VSD and multiple aortopulmonary collaterals who underwent a successful complementary occlusion of a large collateral vessel using an Amplatzer® vascular plug after a previously failed attempt of occlusion with a coil. The percutaneous procedure, performed from the femoral artery before the complete surgical repair, provided an immediate closure of the vessel. This new device is safe and effective for the occlusion of aortopulmonary collaterals, specifically if of large dimensions.

Invited commentary

A combined endovascular and surgical approach is emerging as a potential alternative in the management of high-risk patients with thoracoabdominal aneurysms. A lower incidence of paraplegia may result from reduced visceral ischemia. Mortality, as Black et al reported, may not be significantly different from that seen by those centers with experience performing complete surgical repair. Inherent to its endovascular component, endoleaks may affect its durability. In addition, graft-related issues such as aortoenteric erosion or fistula may be seen with increased incidence, given the necessary extra-anatomic routing for visceral retrograde grafts. It is imperative to protect these grafts with either surrounding tissue or omental flaps to avoid this long-term complication. Until longer follow-up is available, this hybrid procedure should be reserved for high-risk patients who would otherwise be unacceptable for standard repair. In particular, younger patients should be preferentially considered for surgical repair. As further refinements are made to endovascular grafts to reduce early and long-term issues, a combined approach to repair thoracoabdominal aneurysms is likely to gain acceptance. As reported by the authors, a staged approach for patients with aortic dissection may be preferred, assuring adequate inflow for retrograde grafts. Based on their experience, the hybrid procedure had poor results in patients with ruptured aneurysms, probably related to longer operative time. For patients with a prior thoracotomy, a combined approach is an attractive option. Paraplegia, perhaps the most feared complication of open repair, may be reduced. Complete endovascular repair of complex suprarenal and thoracoabdominal aneurysms using branched endografts is in early development. It may take years before this advanced technology is available for routine use. Even then, a combined endovascular and surgical approach may be preferred in some instances and used with more frequency. Vascular surgeons are the principal specialists with the necessary skills and judgment to offer all three alternatives.

Transcatheter closure of a Potts' shunt with subsequent surgical repair of tetralogy of fallot

Closure of a Potts' aortopulmonary anastomosis during repair of a congenital heart malformation has been associated with high morbidity and mortality, especially due to technical problems encountered at closure of the shunt. A case is presented of a 40-year-old patient with tetralogy of Fallot and pulmonary atresia after palliation during infancy with a Potts' shunt. The anastomosis was successfully occluded using an Amplatzer ventricular septal defect occluder, immediately followed by complete surgical repair of his tetralogy of Fallot.

Staged rehabilitation of ductal origin of the left pulmonary artery in an infant fallot's tetralogy

Isolation of a branch pulmonary artery from ductal closure is an unusual finding in patients with tetralogy of Fallot. A case report of a newborn is presented where the closing arterial duct was balloon-dilated and stented to reestablish blood supply to the affected lung as a strategy of improving systemic saturations and promoting the growth of the hiliar branch pulmonary artery. Five months after the initial palliation, complete surgical repair with stent removal was successfully achieved.

Bony density in adolescents after surgical repair of tetralogy of Fallot: a comparative study with healthy adolescents

Adverse influences arising in fetal life or immediately after birth have a permanent effect on body structure, physiology and metabolism. Evidence is now accumulating that programming of bone growth might be an important contributor to the later risk of osteoporosis. Long-term morbidity and mortality associated with retralogy of Fallot is not completely known. The aim of the present study was to evaluate the state of the bones in adolescents after surgical repair of tetralogy of Fallot, so as to ascertain any possible repercussions of the disease on bone mineralization. We studied 34 adolescents with repaired tetralogy of Fallot, between the ages of 11 and 18 years, to establish their nutritional status, in terms of height, weight, and skinfolds, their body composition using an anthropometric method, their sexual maturity according to Tanner, and their food-habits as based on 24-hour recall. Bone density was evaluated by lumbar dual-energy X-ray absorptiometry. We included 34 healthy eutrophic adolescents, matched for gender and age, as controls. No significant differences were observed between the patients and their controls concerning nutritional status, body composition, total energy intake and nutritional supply in macronutrients, calcium, phosphorus, magnesium and vitamin D. Bone mineral density, expressed in Z-score and g/cm2, was significantly higher in patients with tetralogy of Fallot (p < 0.01). The age at the time of the first surgical procedure, or at complete surgical repair, and the total number of surgical procedures, had no significant influence on nutritional status or bone mineralization. Gender, chronological age, sexual maturity and the index of body mass are the major determinants of bone density for both samples. Obese adolescents with repaired tetralogy of Fallot had a significantly higher bone density (p < 0.05) compared to undernourished or euthrophic patients. Being born with tetralogy of Fallot has no significant repercussion, by the stage of adolescence, on nutritional status, pubertal progression, and accretion of bone minerals subsequent to surgical repair. Nutritional status is the major influence on the accretion of bone mass.

Bronchomalacia associated with pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries, and chromosome 22q11.2 deletion.

Respiratory distress is one of the major complications in young infants with pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries (PA-VSD-MAPCA); however, its aetiology remains obscure. We have previously reported an association of bronchomalacia with PA-VSD-MAPCA in patients with a hemizygous deletion of chromosome 22q11.2 (del.22q11). To clarify the clinical relevance of bronchomalacia in patients with PA-VSD-MAPCA and del.22q11, we reviewed the clinical and laboratory records of four patients with PA-VSD-MAPCA who had del.22q11 and bronchomalacia. External bronchial compression by anomalous patterning of the aorta and MAPCA was documented in three of the four patients, using combinatorial examination of angiocardiography, bronchography, fibreoptic bronchoscopy and magnetic resonance imaging. One of the four patients died suddenly of severe respiratory distress at 4 years of age, while the remaining three were inoperable for complete surgical repair. Our study indicates that bronchomalacia as a result of external vascular compression may be an aetiology of early-onset respiratory distress in some patients with PA-VSD-MAPCA and del.22q11, and can significantly affect the clinical outcome.

Complete surgical repair of aortic atresia with a normal left ventricle

Aortic atresia with a normal left ventricle and normal mitral valve is an uncommon congenital lesion. We present two such cases and describe two different approaches of achieving biventricular surgical repair.

Coronary echocardiography in tetralogy of Fallot: diagnostic accuracy, resource utilization and surgical implications over 13 years

OBJECTIVESThis study sought to determine the diagnostic accuracy and impact of the systematic use of coronary echocardiography in a large group of preoperative patients with tetralogy of Fallot (TOF).BACKGROUNDAccurate preoperative identification of an anomalous coronary artery crossing the right ventricular outflow tract (RVOT) in patients with TOF is important to prevent coronary injury during surgical repair.METHODSA retrospective review identified 598 patients with TOF between 1983 to 1995 who underwent an echocardiogram at <2 years old before complete surgical repair. Associated diagnoses included pulmonary stenosis (n = 433), pulmonary atresia (n = 121), common atrioventricular canal (n = 17), absent pulmonary valve syndrome (n = 24) and aortopulmonary window (n = 3).RESULTSBased on intraoperative findings, 32 patients (5.4%) were found to have a major coronary artery crossing the RVOT. The use and diagnostic performance of coronary echocardiography increased over time, while the number of patients undergoing preoperative cardiac catheterization declined. During the most recent study period (1991 to 1995, n = 274), 97% of patients underwent coronary echocardiography yielding a sensitivity of 82%, specificity of 99% and accuracy of 98.5%. Of the 18 patients with TOF and pulmonary stenosis who had abnormal coronary arteries during this period, only 6 (33%) required an extracardiac conduit as part of their complete repair.CONCLUSIONSCoronary echocardiography is an accurate noninvasive tool to delineate coronary anatomy in infants with TOF before complete repair. Routine preoperative cardiac catheterization solely for diagnosis of coronary anatomy is not necessary. The use of an extracardiac conduit can be avoided in the majority of patients with TOF and pulmonary stenosis who have a major coronary artery crossing the RVOT.

Association d'une bronchopathie sévère (moules bronchiques, dilatation des bronches) et d'un retour veineux pulmonaire anormal partiel à un syndrome de Turner chez deux enfants

The prevalence of cardiovascular malformation is high in Turner's syndrome. We report two cases of partial anomalous pulmonary venous drainage associated with a severe bronchial disease. Case 1: a 5-year-old girl with monosomy X presented with acute respiratory failure. The chest X-ray showed an atelectasis of the left lower lobe. The patient improved following bronchoscopic removal of a bronchial cast. During hospitalization, an anomalous pulmonary venous drainage of the right lung was discovered. In spite of complete surgical repair of the cardiac malformation, several episodes of respiratory failure occurred several years after the operation. Case 2: a 9-year-old girl with monosomy X and coarctation of the aorta underwent pre-operative cardiac catheterization. She had a history of bronchiectasis, leading to a lobectomy. Her respiratory condition improved following surgery. A partial anomalous pulmonary venous return was discovered during the cardiac angiography. The coarctation was repaired surgically. The correction of the left-to-right shunt was not necessary. The partial anomalous pulmonary venous return is frequently reported in Turner's syndrome. The possible respiratory disease associated with this cardiac malformation is usually described as a consequence of the increase in pulmonary blood flow. Our two cases did not match this hypothesis.

Effects of modified and classic Blalock-Taussig shunts on the pulmonary arterial tree

Background. The aim of this study was to assess by angiography the late effects of both classic and modified Blalock-Taussig shunts in terms of growth and development of stenosis and distortion. Methods. At a mean of 51 months postoperatively, we retrospectively reviewed the results in 78 patients who underwent creation of Blalock-Taussig shunts (25 classic and 71 modified). Results. At the level of the anastomosis, the shunt caused a reduction in diameter of the pulmonary artery in 49% of all shunts, major stenosis (>50% narrowing of the lumen) in 14%, and distortion of the pulmonary artery in 19%, findings that did not correlate with the type of shunt. Distortion did correlate with younger age at the time of shunt operation ( p = 0.01). Conclusions. After a Blalock-Taussig shunt, growth of the pulmonary arteries occurred but did not exceed the normal growth of the pulmonary arterial tree. Moreover, a shunt procedure can cause distortion and stenosis of the pulmonary artery, which may have important implications for future corrective surgical intervention. All these findings support earlier complete surgical repair of correctable congenital cardiac defects.

An unusual anomalous course of a coronary artery from the pulmonary trunk, coexisting with congenital mitral stenosis and aortic coarctation

AbstractAnomalous origin of a coronary artery is occasionally associated with other congenital heart malformations. Presence of the anomalous artery can then complicate surgical repair. We report an unusually complex arrangement in a patient who died 16 hours following complete surgical repair despite appropriate management.

Response To Human Recombinant Erythropoietin (Epo) in Infants With Congenital Heart Disease And Reduced Pulmonary Blood Flow 16

There is a selected group of infants with Congenital Heart Diseases (CHD) characterized by reduced pulmonary blood flow. In them, arterial oxygen saturation levels (O2Sat) is persistently low (usually around 80%) until complete recovery or surgical repair. These infants require high Hemoglobin(Hb) concentrations and Hematocrit (Hct) levels to preserve adequate arterial content and delivery of O2. They frequently need blood transfusions to maintain Hct levels of at least 55%. Human EPO has been used clinically in infants with anemia of prematurity and bronchopulmonary dysplasia, but there is no clinical experience with its use in newborns with cyanotic CHD. In this observational pilot study we evaluated whether early use of EPO in these infants blunts the physiological descent of Hb that occurs during the first 12 weeks after birth and reduces the need for transfusions. We present preliminary data in 10 infants. Six of them had Pulmonary Stenosis (PS) and poorly compliant right ventricle. They all showed significant improvement in O2Sat after valvular ballon dilation, but O2Sat remained below 86%. The other 4 infants had complex CHD not amenable to complete primary repair during the early neonatal period (i.e. AV canal. TGA with pulmonary stenosis). Infants were included in the study if the Hct was in progressive descent and it was 45%. While on EPO, two patients developed Hct>60%, though post-discharge follow-up and frequency of lab test was inappropiate. We conclude that the early use of EPO abolishes the physiologic descent of Hb and Hct in these infants. These findings may render EPO useful, safe and cost-efficient for the adjuvant management of patients with CHD and reduced pulmonary flow until the condition resolves completely, either spontaneously (i.e. improved compliance of right ventricle in cases of PS), or surgically.

Bilateral branch pulmonary artery obstruction due to kinking at insertion sites of bilateral ductus arteriosus

Bilateral ductus arteriosus (or ligamentum arteriosum) with right aortic arch and isolation of the left subclavian artery is a rare anomaly of the aortic arch system. We report on a patient with complete atrioventricular septal defect, right aortic arch, bilateral ligamentum arteriosum, and isolation of the left subclavian artery in whom kinking at the ductal insertions caused bilateral branch pulmonary artery obstruction. Complete surgical repair was performed when the patient was 4 months of age, and the pulmonary artery obstructions were entirely relieved by ligation and division of both ducts without pulmonary arterioplasty.

Coexisting left congenital diaphragmatic hernia and esophageal atresia with tracheoesophageal fistula: Successful management in a premature neonate

The combination of left congenital diaphragmatic hernia (CDH) with esophageal atresia (EA) and distal tracheoesophageal fistula (TEF) is extremely rare and is considered highly lethal. The authors describe a premature neonate with this association, who is alive at 6 1 2 years of age. Temporary banding of the gastroesophageal junction and gastrostomy was performed concurrently with hernia repair and prosthetic abdominoplasty to enlarge the abdominal cavity. A right thoracotomy for ligation of the fistula, using extracorporeal membrane oxygenation (ECMO), was performed 13 days later. Complete repair of the esophageal atresia was accomplished 7 weeks after birth. The methods that have been suggested in the literature are discussed. The institution of ECMO at birth could allow a primary complete surgical repair of EA and CDH. Nevertheless, surgical management with staged repair, as described herein, can be useful.

Tetralogy of fallot with diminutive pulmonary arteries: Preoperative pulmonary valve dilation and transcatheter rehabilitation of pulmonary arteries

Objectives. The study sought to determine the results of a novel transcatheter management approach in tetralogy of Fallot with diminutive pulmonary arteries.Background. Tetralogy of Fallot with diminutive pulmonary arteries and severe pulmonary stenosis is rare and resembles tetralogy of Fallot with pulmonary stresia: There is a high incidence of aortopulmonary collateral channels, arborization abnormalities, stenoses and need for multiple operations. Because a combined catheter-surgery approach facilitates repair in these patients, such an approach may benefit those with diminative pulmonary arteries and pulmonary stenosis.Methods. Clinical, catheterization and surgical data were studied retrospectively for 10 such patients undergoing preoperative pulmonary valve balloon dilation, among other transcatheter interventions, from January 1989 to January 1995.Results. Initially, the Nakata index ranged from 20 to 98 mm2/m2(mean 67 ± 28 mm2/m2). The pulmonary valve was first balloon dilated (mean balloon/annulus 1.5 ± 0.3), and the mean initial valve annulus Z score (−40 ± 1) increased to −3.3 ± 1.1 (p < 0.01). Other interventions included branch pulmonary artery balloon dilation (7 patients, 23 vessels) and coil embolization of aortopulmonary collateral channels (8 patients, 31 collateral channels). At preoperative follow-up catheterization, the mean pulmonary annulus Z score was −3.1 ± 0.7, and the aorta index increased to 143 ± 84 mm2/m2(p < 0.03). All patients underwent complete surgical repair successfully. At a mean follow-up period of 2.6 ± 2 years, right ventricular pressure was < 70% systemic in all patients and <50% systemic in seven.Conclusions. In patients with tetralogy of Fallot, severe pulmonary stenosis and diminutive pulmonary arteries, initial pulmonary valve balloon dilation increases the annulus Z score and anterograde pulmonary blood flow and facilitates simultaneous coiling of sertopulmonary collateral channels and access for branch pulmonary artery dilation, all of which results in pulmonary artery growth, simpligying surgical management.

Transposition of the great arteries {S, D, L}: Pathologic anatomy, diagnosis, and surgical management of a newly recognized complex

The transposition of the great arteries {S,D,L} complex is delineated for the first time from the anatomic, diagnostic, and surgical standpoints in this study of 26 cases: 16 surgical and 10 postmortem. Transposition of the great arteries with situs solitus of the viscera and atria (S), D-loop ventricles (D), and L-transposition (L) was characterized by six additional interrelated anomalies that largely determined surgical management: (1) ventricular septal defect, usually conoventricular, in 96%; (2) malalignment of the conal septum, typically leftward and posteriorly, in 80%; (3) right ventricular hypoplasia in 50%; (4) pulmonary outflow tract stenosis in 27%; (5) ventricular malposition, such as superoinferior ventricles, in 23%; and (6) absent left coronary ostium resulting in “single” right coronary artery in 23%. Complete surgical repair was done in 81% of the surgical patients with a 12.5% hospital mortality rate and no late deaths. When there was no pulmonary outflow tract stenosis and intracardiac anatomy was uncomplicated, we undertook anatomic repair before 1 month of age. However, when pulmonary outflow tract stenosis coexisted, complete repair was deferred until after age 1 year, our currently preferred operation being the REV procedure (réparation à l'étage ventriculaire). When complex intracardiac anatomy precluded biventricular repair, a palliative procedure was performed in 19% without mortality. Hence, this experience indicates that surgical management of patients with the transposition of the great arteries {S,D,L} complex is feasible. (J T HORAC C ARDIOVASC S URG 1995;110:613-24)

Profile of paediatric patients with pulmonary hypertension judged by responsiveness to vasodilators.

To describe the demographic and haemodynamic variables of children presenting with primary pulmonary hypertension or pulmonary hypertension appearing or persisting after surgical correction of congenital heart defects and to assess the acute effect of vasodilator drugs on their pulmonary vascular bed. Retrospective review. Paediatric cardiology department and intensive care unit of a large tertiary centre. Fourteen consecutive patients presenting with primary pulmonary hypertension (group 1) or pulmonary hypertension persisting or appearing late after complete surgical repair (group 2). Baseline haemodynamic measurements were taken in room air at rest and repeated in 100% oxygen. With constant monitoring of heart rate and pulmonary and systemic arterial pressures, patients were given serial intravenous, sublingual, or oral incremental doses of vasodilators (mean 4.1 trials per patient). The maximum effect of the drug was charted. A positive response to acute vasodilator tests was defined as a decrease in mean pulmonary or mean systemic arterial pressure > 15% with the mean pulmonary artery pressure not reaching the systemic level and either no change or an increase in mean systemic arterial pressure. Haemodynamic variables between groups (1 v 2, responders v non-responders, patients experiencing or not experiencing adverse effects to vasodilators) were compared by a two tailed unpaired Student's t test, and their survival curves were compared by the log rank statistic. Groups are small and definitive conclusions are difficult to draw, but the baseline haemodynamic assessments were not significantly different between group 1 and 2 or between responders and non-responders to vasodilators. Patients experiencing adverse effects had a higher pulmonary vascular resistance (p = 0.04) and wedge pressure (p = 0.02) than those without adverse effects. Of the vasodilators used, tolazoline, hydralazine, salbutamol, phentolamine, and phenoxybenzamine were ineffective. A positive response was seen in five of 13 patients given oxygen, in one of eight given prostacyclin, four of 12 given nifedipine, four of eight given diltiazem, one of six given captopril, and two of seven given glyceryl trinitrate. Estimates of survival of the population with primary pulmonary hypertension were 37% at one year and 12% at 2.5 years. Survival was significantly shorter in the non-responders than in the responders (p = 0.005). Children with primary pulmonary hypertension present to the cardiologist at a young age (five of eight were younger than 7 years) but with advanced pulmonary vascular disease and have a poor prognosis. 64% of group 1 and group 2 patients had a positive response to acute treatment with at least one vasodilator. Calcium channel blockers were the most effective agents. There was a positive response to drugs despite a negative response to acute treatment with oxygen. The survival of non-responders was shorter than that of the responders.

Laryngotracheoesophageal cleft (type IV): Management and repair of lesions beyond the carina

Since the first successful repair of a complete laryngotracheoesophageal cleft (LTEC) to the carina in 1982, three newborn infants were observed with a particularly difficult variant in which the cleft extends beyond the carina into the mainstem bronchi. This type IV LTEC creates a long common tracheoesophagus, whose successful separation requires meticulous preoperative, operative, and postsurgical care. Three infants had complete surgical repair at our institution at 29, 49, and 225 days old and survived a minimum of 8 months. Recurrent tracheoesophageal fistulae at the thoracic inlet occurred in two infants, but was not observed in one patient when sternocleidomastoid muscle was interposed between the trachea and esophagus in the neck. Microgastria is an associated finding in each infant with the tracheoesophageal cleft extending beyond the carina. The small stomach is problematic as it is anatomically inadequate for any antireflux procedure and has not grown well, even with prolonged feeding. Early cleft repair is essential to prevent the development of chronic lung disease secondary to recurrent aspiration. The techniques to make the diagnosis, the preferred treatment to initially protect the airway, a single-stage operation performed simultaneously through the chest and neck to definitively repair the cleft, and finally the intraoperative and postoperative management critical for an optimal outcome are described.

Natural and modified history of complete atrioventricular septal defect--a 17 year study.

We reviewed 103 cases of isolated complete atrioventricular septal defect. These cases represented 4.4% of the cases of congenital heart disease diagnosed in our hospital by catheterisation and angiography during 1971-88. Most children (n = 76) had Down's syndrome. Banding of the pulmonary artery was performed in seven cases and complete repair in 67 cases. In the period 1971-82 the complete correction was performed at a mean age of 23 months with a surgical mortality of 88.8%. In the period 1983-8 the mean age at complete correction was 13 months, the mortality 43.2%, and the five year actuarial survival was 46.8%. The 22 patients that survived after complete correction were in functional classes I and II of the New York Heart Association classification. After a mean follow up of 10 years only eight (36%) of the 22 who were followed up and treated medically survived; all had developed pulmonary vascular obstructive disease and were in functional classes III or IV. Our findings stress the importance of early complete surgical repair.