Major Associated Cardiac Anomalies Research Articles

Total anomalous pulmonary venous connection with another major congenital cardiac defect in biventricular hearts.

The association of total anomalous pulmonary venous connection (TAPVC) with major congenital cardiac anomalies is rare. This coexistence has an impact on the diagnosis and management strategies. The objective of this single centre study was to analyse the outcomes of such association in bi-ventricular hearts. This is a single-centre retrospective review of all patients who underwent repair for TAPVC associated with major congenital cardiac anomalies in bi-ventricular hearts from February 2015 to March 2020. Data were obtained from the hospital database. The primary aim was to assess the surgical outcomes. In addition, we described the incidence, pathophysiology, morphology and surgical strategies in this cohort of patients. Out of a total of 274 patients who underwent repair for TAPVC during the study period, 16 (5.8%) patients had one or more major associated cardiac defect anomalies. The major associated lesions were ventricular septal defects (9), tetralogy of Fallot (2), transposition of the great arteries (1), aorto-pulmonary window (1), partial atrioventricular canal defect (1), pulmonary stenosis (1) and severe tricuspid valve regurgitation due to flail chord (1). The mean follow-up period is 32 ± 13 months. There were two deaths in the post-operative period and no deaths in the follow-up period. The incidence of major associated cardiovascular defects with TAPVC in bi-ventricular morphology is 5.8%. The presence of a major associated lesion with TAPVC significantly alters the pathophysiology and clinical presentation with an impact on diagnosis and treatment.

Abstract 9008: Echocardiographic Markers in Early Postnatal Life to Predict Intervention in Coarctation of Aorta

Background: Coarctation of the aorta (CoA) is challenging to diagnose in the early neonatal life due to the patent ductus arteriosus (PDA). We aimed to describe the resource utilization, early post-natal cardiac profile and predictors for need of cardiac surgery. Methods: Retrospective study of infants with an antenatal suspicion of CoA, without major associated cardiac anomalies, born at ≥37 weeks during 2014 to 2020. The analyzed echocardiography (ECHO) was performed within 48 hours after birth. Those not requiring post-natal intervention (normal group) were compared to those that required post-natal cardiac surgery (CoA). Strain was measured using speckle-tracking ECHO (STE). Results: A total of 51 newborns were included, of which 40 (78%) were normal and 11 (22%) underwent intervention. In the normal group, median hospitalization was 4 [2-6] days, 6 (15%) were exposed to prostaglandin E (PGE) infusion, 38 (95%) were on intravenous fluids, 9 (23%) required gavage and age at full feeds was 3 days (IQR: 2-5). Ductus was patent in all newborns and aortic arch measurements were significantly smaller in the CoA (ascending [p=0.01], proximal, distal, isthmus [ p <0.001]). Right ventricular (RV) predominance was higher in the CoA group, as demonstrated by the left ventricular (LV) end-systolic eccentricity index (EI) (1.60 [0.28] vs 2.16 [0.45]; p <0.001) and by a larger end-diastolic area (EDA) of the RV in apical 4 chamber (A4C) relative to the LV-EDA - with a ratio of 1.02 [0.2] vs 1.56 [0.23]; p <0.001. Isthmus and EI were predictive for intervention (OR 26, 95%CI (-42 to -9); p =0.002, OR 4.5, 95%CI (1.5-7.5); p =0.003). A RV to LV EDA ratio in A4C greater than 1.3 demonstrated a high probability for intervention (AUC = 0.97). CoA newborns had a lower RV deformation by STE (peak systolic strain rate (SR); -0.98 [0.17] vs -0.83 [0.2]; p =0.02, early diastolic SR; 1.05 [0.2] vs 0.85 [0.26]; p =0.01). Conclusions: Most infants with antenatal suspicion of CoA did not require intervention but were high consumers of resources. Despite PDA patency, isthmus and RV predominance on first post-natal ECHO were predictors for surgery. The novel markers of strain by STE and ventricular EDA ratio in A4C may help predicting those with true CoA requiring intervention.

Risk factors for recoarctation and results of reoperation: a 40-year review.

We analyzed a single institution 40-year experience with children that had coarctation repair to define risk factors for recoarctation and to evaluate the results of reoperation for recoarctation. Between 1957 and 1998, 271 patients had primary surgical repair of coarctation of the aorta. Techniques for primary repair included Gore-tex patch aortoplasty (PATCH; n = 118), resection with extended end-to-end anastomosis (RXEEA; n = 69), subclavian flap aortoplasty (SFA; n = 61), resection with simple end-to-end anastomosis (ETE; n = 18), resection and interposition graft (n = 4), and extra-anatomic graft (n = 1). Techniques for recoarctation repair included PATCH, interposition graft, and extra-anatomic graft. Median age at initial repair was 156 days. Major associated cardiac anomalies were present in 96 patients (35%). A hypoplastic aortic arch was present in 37 patients (14%). There were three early deaths (1%) and six late deaths (2%). One patient had paraplegia (0.4%). Recoarctation occurred in 29 patients (11%) and was most frequent with ETE and SFA repairs (33% and 20%, respectively). Multiple logistic regression analysis revealed ETE repair (p = 0.0002), SFA repair (p = 0.049), and aortic arch hypoplasia (p = 0.0001) to be risk factors for recoarctation. Using PATCH as the covariate, the odds ratio to develop recoarctation was 3.5 for SFA, 17.2 for ETE, and 15.2 for hypoplastic aortic arch. There was no mortality or paraplegia after recoarctation repair (n = 23). Six patients had seven balloon angioplasties; two of these patients later required reoperation. Three patients required a second reoperation for persistent coarctation. In our 40-year review, simple end-to-end anastomosis and subclavian flap repair had the highest incidence of recoarctation, especially when associated with aortic arch hypoplasia. We recommend resection with extended end-to-end anastomosis for repair of neonates and infants and Gore-tex patch aortoplasty for children over the age of 1 year. Surgical repair of recurrent coarctation is safe, effective, and has a low incidence of persistent coarctation.

Risk factors associated with mortality and interventions in 472 neonates with interrupted aortic arch: A Congenital Heart Surgeons Society study

We sought to determine the prevalence of outcomes and associated patient and management factors for neonates with interrupted aortic arch. From 1987 to 1997, a total of 472 neonates were enrolled prospectively from 33 institutions. Competing risks methodology was used to determine simultaneous risk and associated incremental risk factors for death, initial and subsequent left ventricular outflow tract procedures, and arch reinterventions. Overall survival was 59% at 16 years after study entry but improved with successive birth cohort. In general, risk factors for death in each of the competing risks analyses included lower birth weight, younger age at study entry, type B interrupted aortic arch, and major associated cardiac anomalies. Of 453 patients who had interrupted aortic arch repair, after 16 years 33% had died and 28% had undergone an arch reintervention. Reintervention was more likely for those who had truncus arteriosus repair, interrupted aortic arch repair by a method other than direct anastomosis with patch augmentation, and the use of polytetrafluoroethylene as either an interposition graft or a patch. From study entry, competing risks after 16 years showed that 28% had died and 34% had undergone an initial left ventricular outflow tract procedure. Initial left ventricular outflow tract procedure was more likely for those with single ventricle, type B interrupted aortic arch, bicuspid aortic valve, or anomalous right subclavian artery. Among those who had undergone an initial left ventricular outflow tract procedure, after 16 years 37% had died and 28% had undergone a second procedure. Anatomic features affect mortality and initial left ventricular outflow tract procedures, whereas characteristics of the arch repair affect arch reintervention.

Anatomically corrective repair of complete atrioventricular septal defects and major cardiac anomalies

Background. Although satisfactory results of corrective surgery for atrioventricular septal defects (AVSDs) with complex lesions such as double-outlet right ventricle (DORV) or atrial isomerism have been reported in recent years, the optimal surgical options for isomerism hearts is still a controversial issue. Methods. We performed anatomically corrective repair on 13 children with balanced forms of complete AVSDs and associated major cardiac anomalies. Eight of the 13 patients had atrial isomerism (right in 1, left in 7), 5 of whom had DORV. Four others had DORV with trisomy 21, and 1 had tetralogy of Fallot. Atrial septation for isomerism with the placement of an additional prosthesis was performed on 4 patients. Results. Two hospital deaths and one late death occurred only in the isomerism group. Three reoperations were required solely in the left isomerism group: one replacement of the valved external conduit concomitantly with reconstruction of the left ventricular outflow obstruction, one mitral valve replacement for severe regurgitation of left atrioventricular valve, and one relief of progressing left ventricular outflow obstruction. Conclusions. The results of anatomically corrective surgery for AVSDs with major associated cardiac anomalies in the nonisomerism group were excellent. The optimal surgical options for isomerism heart, however, remain a controversial issue.

Aortopulmonary window associated with complete atrioventricular septal defect

Aortopulmonary (AP) window and atrioventricular (AV) septal defect are congenital cardiovascular defects that typically occur without major associated cardiac anomalies but in a minority of cases are found in conjunction with a variety of other lesions. We describe the case history of an infant with coexisting AP window and complete AV septal defect who underwent complete primary repair at 3 months of age. To our knowledge, this combination of congenital cardiovascular defects has not previously been described. Clinical summary. The patient was born at 37 weeks’ estimated gestational age by spontaneous vaginal delivery, with a birth weight of 3.0 kg and Apgar scores of 8 at 1 minute and 9 at 5 minutes. At 2 months of age, she was referred for cardiac evaluation because of a cardiac murmur and failure to thrive. She weighed 3.4 kg and was feeding poorly. On evaluation, she was found to be nondysmorphic. Cardiac examination disclosed a grade 3/6 holosystolic murmur, a right ventricular heave, a diastolic rumble over the apex of the heart, and a gallop. The oxyhemoglobin saturation was 97% on room air, and the heart rate and blood pressure were within the normal ranges for age. The electrocardiogram showed a normal sinus rhythm, left axis deviation, right atrial enlargement, biventricular hypertrophy, and nonspecific changes in the ST segment. The echocardiogram demonstrated a Rastelli type C complete AV septal defect with large atrial and ventricular components and moderate regurgitation of the left-sided component of the common AV valve, along with a left-sided superior caval vein draining to the right atrium via an enlarged coronary sinus. The patient was initially managed medically with digoxin and furosemide, but there was little improvement in her growth. At the age of 3 months, a repeat echocardiogram confirmed the prior diagnosis but also revealed a large AP window between the proximal pulmonary trunk and ascending aorta, with continuous left-to-right shunting. Cardiac catheterization was performed to assess her hemodynamic

Complete atrioventricular septal defects: the influence of associated cardiac anomalies on surgical management and outcome

Major associated cardiac anomalies are known to increase the risk of repair of complete atrioventricular septal defects (CAVSDs). The purpose of this study was to examine the effects of such anomalies on the current surgical management of CAVSDs and their influence on outcome following repair. We performed a retrospective review of a 100 consecutive non-isomeric patients undergoing repair of CAVSD at our institution, between January 1989 and December 1994; patients with partial or intermediate defects were excluded. Complete atrioventricular septal defect patients with other major cardiac abnormalities (complex) were then compared to those with isolated CAVSDs. There were 15 patients (15%) with associated anomalies; 3 had tetralogy of Fallot, 1 patient had pulmonary atresia, 6 had hypoplastic left or right ventricle, 1 had tetralogy of Fallot and hyperplastic right ventricle, 2 patients had double outlet right ventricle, 1 had hypoplastic aortic arch and 1 patient had aortic coarctation. The median age at operation was similar for both groups (4.2 months), while the median weight was not significantly different for isolated CAVSDs compared to complex (4.2 months vs 3.4 months, P = 0.89), but there was a higher incidence of trisomy 21 (70/85, 82% vs 8/15, 53.3%, P = 0.01). Two of the 85 isolated CAVSD patients (2.3%) had undergone palliative pulmonary artery banding, while 5 of the 15 complex patients (33.3%) had either banding or Blalock-Taussig shunts performed. The technique of CAVSD repair was identical in each group. All complex patients had standard repair of their associated anomalies. Hospital mortality was higher in the complex group (3/15, 20% vs 2/85, 2.3%, P = 0.004); all early deaths in the complex group occurred in patients with a hypoplastic ventricle. Reoperation for left atrioventricular valve regurgitation was required in six isolated CAVSD patients (7.1%) and in one complex (6.6%). In the absence of significant ventricular hypoplasia, the early results of surgical repair in patients with CAVSDs and associated cardiovascular anomalies are similar to those achieved in patients with isolated CAVSD.

Early and long-term results for correction of total anomalous pulmonary venous drainage (TAPVD) in neonates and infants

To present our 17-year experience of surgical repair of total anomalous pulmonary venous drainage (TAPVD) in 71 consecutive neonates and infants, with particular emphasis on the role of preoperative pulmonary venous obstruction (PVO), the management of postoperative pulmonary hypertensive crises and the long-term results. From July 1977 to May 1994, 36 newborns and 35 infants, aged between 1 and 300 days, had repair TAPVD in our Department. Fifty-four patients had decompensated heart failure, whereas three were moribund. The anomalous drainage was supracardiac in 32 (45%), cardiac in 17 (24%), infracardiac in 17 (24%) and mixed in 5 infants (7%). Major associated cardiac anomalies coexisted in nine cases. Forty-five (63%) exhibited signs of PVO. The repair was performed under moderate hypothermia in 15 and deep hypothermic arrest in 56 patients. In case of postoperative pulmonary hypertensive crises, artificial hyperventilation with high oxygen concentration, sedation and relaxation, inotropic support and afterload reduction with phentolamine were applied selectively. The actual follow-up is based on functional symptomatology and cardiac echo-doppler studies. Six children (8.5%) died early: four perioperatively, two on the 2nd and 10th postoperative day, respectively. Poor preoperative condition, operation before 1987 and preoperative PVO were the main incremental risk factors for death. No death occurred among the last 38 cases. There were complications in 45 patients, in particular pulmonary hypertensive crises in 27. Two patients died late, after 80 and 118 days, respectively, of progressive intrapulmonary vein fibrosis: the second despite reoperation. The risk of late death approaches zero after 6 months. Among the 63 survivors, 56 (86%) are asymptomatic and without medication, 3 months-17 years after operation. Five have mild symptoms with ordinary physical activity. Two are lost to follow-up. Early repair of TAPVD with aggressive management of pulmonary hypertensive crises carries low operative mortality nowadays. Preoperative PVO as a risk factor has been neutralized since 1987. Long-term results are gratifying: no late death after 6 months, no reoperation and functional good results. Progressive pulmonary vein fibrosis remains an unpredictable rare cause of death within the 1st year after surgery.

Cor Triatriatum: Diagnosis, Operative Approach, and Late Results

From May 1960 to January 1992, 13 patients with cor triatriatum underwent surgical correction at the Mayo Clinic. Their ages ranged from 7 months to 57 years. Four patients had isolated cor triatriatum; three others had an associated patent foramen ovale or atrial septal defect. Six patients had major associated cardiac anomalies. In patients who had isolated cor triatriatum or associated anomalies with high pulmonary blood flow, the mean gradient across the left atrial diaphragm was 24.4 mm Hg, in comparison with 11.6 mm Hg in patients who had cor triatriatum and a connection between the common pulmonary venous chamber and the right atrium or associated cardiac anomalies that restricted pulmonary blood flow. In all 11 patients who underwent angiography, echocardiography, or both at our institution, the correct diagnosis was established preoperatively. Currently, echocardiography is the procedure of choice for diagnosing cor triatriatum. The diaphragm was excised through a left atrial approach in seven patients and through a right atriotomy in six. All associated anomalies were corrected. One critically ill patient who underwent an emergency operation in 1963 died early postoperatively, and one patient with chromosomal abnormalities and multiple cardiac defects died 2 months after an uneventful postoperative course. At the time of follow-up of the 11 long-term survivors, 9 were in New York Heart Association functional class I, and 2 (both of whom had major associated cardiac anomalies) were in class II. Postoperative angiography or echocardiography (in nine patients) showed no residual interatrial shunt or recurrent left atrial diaphragm.

Aortopulmonary Window: Factors Associated With Early and Late Success After Surgical Treatment

Between 1953 and 1990, 19 patients, who were from 7 weeks to 27 years old, underwent surgical correction of aortopulmonary window at the Mayo Clinic. Associated cardiac anomalies were present in nine patients (47%). At operation, extracorporeal circulation was used in all except one patient. In seven patients, division and primary closure were done. In four patients, the defect was closed by direct suture through a transpulmonary approach. In eight patients, the defect was closed with a patch through a transpulmonary or transaortic approach. Four deaths (21%) occurred intraoperatively or immediately postoperatively. All four patients had undergone division of the aortopulmonary window before 1962, and three of them had a pulmonary vascular resistance (Rp) that was 7.3 U.m2 or more and a ratio of Rp to systemic vascular resistance (Rp/Rs) that exceeded 0.6. One patient with an Rp of 11.8 U.m2 and an Rp/Rs of 0.72 died 16 years postoperatively. Statistical analysis of risk factors indicated that early year of operation (P = 0.022), division of the aortopulmonary window versus transaortic or transpulmonary closure (P = 0.009), and a high Rp/Rs (P = 0.021) were significantly associated with perioperative death. All patients with a preoperative Rp of 8 U.m2 or less, an Rp/Rs of less than 0.4, and no major associated cardiac anomalies were in functional class I (New York Heart Association) postoperatively. Our study confirms that infants with aortopulmonary window should undergo operation early, before irreversible pulmonary vascular changes have developed. Patients with an Rp/Rs that exceeds 0.4 should be thoroughly assessed to determine their operability.

Staged repair of interrupted aortic arch and ventricular septal defect in infancy

Staged repair of interrupted aortic arch and ventricular septal defect was carried out in 20 infants from 1979 through 1990. Among the important associated cardiac defects were transposition of the great arteries, truncus arteriosus, and anomalous origin of the right pulmonary artery. The first stage, usually consisting of the placement of an 8- or 10-mm polytetrafluoroethylene graft, pulmonary artery banding, and ligation of the patent ductus arteriosus, resulted in 20 survivors (100%) There were two interim deaths (10%) before the second stage of ventricular septal defect closure and pulmonary artery band removal, which had 15 survivors (83%, 15 / 18 ). Because the major morbidity and mortality early in this experience could be traced to leaving the pulmonary artery band on too long, early removal (within 2 to 3 months) was begun. Since 1985, 8 (100%) of 8 infants have survived both stages and are now doing well. Because of the relatively large polytetrafluoroethylene graft, only 1 child (aged 9 years) has experienced substantial late aortic arch obstruction and undergone placement of an 18-mm Dacron graft without difficulty. Of interest is the finding that in only 1 (5%) of the 20 patients has major (≥40-mm Hg gradient) left ventricular outflow tract obstruction developed. In summary, the staged repair of interrupted aortic arch with ventricular septal defect has become very reliable despite the condition of the infant or major associated cardiac anomalies and can be recommended for infants at high risk for primary repair. More long-term information will be needed to determine which approach will be the best choice for the majority of infants.

Repair of Complete Atrioventricular Canal: 15 Years' Experience

Corrective surgery for complete atrioventricular canal was performed over a 15-year period on 72 patients, 49 of whom had Down's syndrome: 46 were without major associated cardiac anomalies and 15 had previously undergone pulmonary artery banding. The pressures in the right and left ventricles equilibrated in 77% of the patients. The early mortality rate was 18% and the late mortality 7%. At follow-up 4% of the patients had severe mitral incompetence and 6% had severe tricuspid incompetence. The mean reduction of pulmonary artery pressure was 40 mmHg. The mean peak systolic pressure ratio between pulmonary artery and aorta was 0.73 before repair and had fallen to 0.38 at follow-up catheterization, when 88% of the patients were asymptomatic. Early repair in the first year of life is nowadays preferred in order to avoid progressive pulmonary vascular disease. Although the alternative of first-stage pulmonary artery banding gave lower (13%) mortality at the corrective operation, it cannot be recommended if atrioventricular valvular incompetence is significant.

Corrective Repair of Complete Atrioventricular Canal Defects and Major Associated Cardiac Anomalies

Twenty-nine patients with balanced forms of complete atrioventricular (AV) canal defects and tetralogy of Fallot in 12, double-outlet right ventricle (DORV) in 10, and additional anomalies associated with atrial isomerism in 7 underwent corrective repair. Age ranged from 39 days to 24 years (median, 3.7 years). Repair included reconstruction of the common AV valve in 25 patients and prosthetic valve replacement in 4. Construction of an intraventricular tunnel connecting the left ventricle with the aorta was accomplished in 23 of 25 patients with ventriculoarterial concordant or double-outlet connections. In 2 patients with situs inversus and associated DORV and in 4 patients in the isomerism group who had ventriculoarterial discordant connections, repair included intraatrial venous diversion by a modified Senning procedure in 2, a modified Mustard venous switch in 1, and a tailored atrial baffle in 3. A valved extracardiac conduit was required for repair in 4 patients. Partial anomalous systemic venous connection was repaired in 7 patients and total anomalous pulmonary venous connection, in 5. There was 1 hospital death (3%) and 3 late deaths (11%). There was 1 (3.6%) reoperation to replace an incompetent right (systemic) AV valve 17 months postoperatively, and each of the 25 surviving patients was in New York Heart Association Functional Class I (80%) or II (20%) at follow-up.

Intermediate-term results of the endocardial surgical approach for anomalous atrioventricular bypass tracts

Between October 1974 and March 1, 1985, 26 patients with 29 anomalous AV bypass tracts underwent surgical treatment at the Medical Center of UAB, with follow-up from 1 to 14 years. Eleven of the patients underwent surgical correction of major associated cardiac anomalies. One patient had His bundle ablation, and 25 patients had direct surgical division of 28 bypass tracts with the use of an endocardial approach. Successful division was achieved in 27 (96%; confidence limits 88% to 99%) of 28 bypass tracts identified pre- and intraoperatively. There were no hospital deaths, but on formal follow-up there were three late deaths, all occurring in patients with major associated cardiac pathology. Neither preexcitation nor reciprocating tachycardia recurred, and functional status was excellent among patients without associated cardiac lesions. Because of the safety and, at least on intermediate-term follow-up, apparently curative nature of this operation (particularly for patients without other major cardiac pathology), surgical treatment is recommended for patients with bypass tracts having lethal potential, those with reciprocating tachycardia unresponsive to drugs, and to symptomatic patients facing many years of medications.

Current results of management in transposition of the great arteries, with special emphasis on patients with associated ventricular septal defect

Two hundred forty-five patients less than 15 days of age with transposition of the great arteries with or without a ventricular septal defect or pulmonary stenosis were entered into an ongoing 20 institution treatment study between January 1, 1985 and June 1, 1986. Complete follow-up is available on all patients. The ventricular septal defect narrowed in only 1 of 36 patients with combined transposition of the great arteries and ventricular septal defect; pulmonary stenosis developed or worsened in 3 of these 36 patients and in 3 of the 187 patients with simple transposition. Twelve month overall survival among the 245 patients was 80%. No morphologic feature of transposition was a risk factor for death but major associated cardiac and noncardiac anomalies (more common in patients with combined transposition and ventricular septal defect) and low birth weight were risk factors. Neither arterial switch repair (n = 86), atrial switch (Mustard) repair (n = 21) nor atrial switch (Senning) repair (n = 39) was a risk factor for death, but results in all surgical groups were better in the last part of the experience. Death before repair was less frequent late in the study. Possibly, in low birth weight infants, survival was better with the arterial than with the atrial switch repair. These data suggest that survival at 1 year is similar with either the arterial or the atrial switch repair. The early results of repair of combined transposition of the great arteries and ventricular septal defect are as good as those of simple transposition. Special institutional efforts are required to attain good results with the arterial switch repair and to prevent death before repair.

The repair of atrioventricular septal defects in infancy

Two (3%) of 72 patients undergoing repair of atrioventricular septal defects, 1984–September 1985, died in hospital, and among the 58 patients with isolated atrioventricular septal defect, one (2%) died in hospital. One (3%) of the 30 patients with isolated atrioventricular septal defects undergoing repair in the first year of life died. The absence of an incremental risk of young age is a continuation of the trend demonstrated in a parametric analysis of the earlier 1967–1982 experience ( n = 310). The hospital mortalities were similar in 1984–September 1985 after repair of partial and complete atrioventricular septal defects, again a continuation of the earlier trend as is the lessened hospital mortality (7%) in the current era among patients with major associated cardiac anomalies. The continuity of results in the two eras, and the predictability of the results in the current era from equations developed from the earlier one, give a high degree of confidence in the inference that primary repair in the first year of life is advisable for most patients with atrioventricular septal defects. Ten-year survival of such patients operated upon currently is predicted to be 99 and 95% for partial and complete atrioventricular septal defects, respectively.

Infant Coarctation: A Spectrum in Clinical Presentation and Treatment

Between 1975 and 1985, 125 infants 2 to 365 days old (majority, 30 days old or less) with coarctation of the aorta underwent surgical repair. Forty-seven patients (38%) had severe congestive heart failure (CHF), metabolic acidosis, and poor systemic perfusion. The predominant operative technique was synthetic patch aortoplasty (100 patients); the remaining 25 had an end-to-end anastomosis. There were no operative deaths. Perioperative complications were minimized with the synthetic patch technique (less than 15%). For patients surviving at least 3 months after repair, the arm-leg systolic blood pressure gradient was relieved in 82% (71/87) of the patients having patch aortoplasty versus 65% (15/23) of the patients with end-to-end anastomosis. Although the rate of reoperation between the two groups was similar (patch, 5 [6%]; end-to-end, 3 [13%], two of the reoperations in the patch group were for preexisting hypoplastic transverse aortic arch. Late deaths (20 patients, 16%) were due to other major associated cardiac anomalies. Patch aneurysms have not occurred. Expedient use of synthetic patch aortoplasty has decreased perioperative complications, relieved coarctation gradients for CHF, increased early survival even in the presence of complex or associated cardiac anomalies, and has an acceptable rate of recurrent coarctation (6 to 13%).

Early and intermediate-term (10-year) results of surgery for univentricular atrioventricular connection (“single ventricle”)

One hundred sixteen patients with "single ventricle" underwent 147 operations between 1967 and July 1982, with an actuarial survival rate (hospital deaths are included in all actuarials) at 10 years of 66%. One hospital death (2%) occurred after 45 primary "classic" shunting operations, and the 10-year actuarial survival was 85%. There were no hospital deaths after atrial septectomy or pulmonary artery banding, and the 10-year actuarial survival rate after these as initial procedures was 76% and 74%, respectively. Thirteen of 36 patients (36%) who underwent ventricular septation between 1967 and November 1, 1983, died in hospital. Among the 13 with the ideal morphologic characteristics of double inlet left ventricle without major associated cardiac anomalies, and without the need for valve replacement or a valved extracardiac conduit, there were no hospital deaths and a late survival rate of 77% (confidence limits 59 to 90%). Sixteen of 73 patients (22%), including those with tricuspid atresia, undergoing the Fontan-type procedure between 1967 and November 1, 1983, died in hospital. Multivariate analysis indicates that cardiac morphologic patterns other than tricuspid atresia are risk factors for hospital death in this group. The 10-year actuarial survival rate among the 73 patients was 71%, but only 1 death (associated with reoperation) occurred more than 2 months after operation. Ninety-seven percent of all surviving patients are in New York Heart Association functional class I or II, as are 100% of those who underwent septation and 96% of those who underwent the Fontan-type procedure.

Management of critical aortic stenosis in infancy

Critical aortic valvular stenosis presents in infancy with severe congestive heart failure. Clinical assessment and electrocardiography are of value, but cardiac catheterization with angiography has been considered mandatory prior to surgical treatment. With cross-section echocardiography an accurate diagnosis of aortic stenosis and associated lesions is possible. Over the past 2 years, we have established a protocol according to which, if a clinical diagnosis of critical aortic stenosis is confirmed by cross-sectional echocardiography in the absence of major associated cardiac anomalies, infants are submitted for aortic valvotomy under inflow occlusion without invasive studies. This protocol was used in an effort to decrease the mortality rate by avoiding the preoperative stress of cardiac catheterization and angiography, as well as the hazards of cardiopulmonary bypass in the severely ill infant. Eight infants with critical aortic stenosis have been operated upon, five without prior cardiac catheterization. Ages at operation ranged from 2 days to 7 months, with six children less than 2 weeks of age. The noninvasive diagnosis was confirmed at operation in each case. There was one early postoperative death and one late death. No death has been related to the technique of inflow occlusion. A decision tree for the noninvasive assessment of suspected critical aortic stenosis based on the clinical features and echocardiographic findings is presented.

Surgical results and protocols in the spectrum of tetralogy of Fallot.

Between 1967 and July 1982, 1103 operations were performed for the tetralogy of Fallot of all types with 116 (10.5%) hospital deaths. Eighty-eight hospital deaths (10.5%) occurred in the 836 patients undergoing repair. The incremental risk factors for hospital death after repair include pulmonary arterial problems (p = 0.0002), major associated cardiac anomalies (p less than 0.0001), small size (young age) (p less than 0.0001), and more than one previous operation (p = 0.0004). Absent pulmonary valve is a risk factor (p = 0.04). In patients with pulmonary stenosis, the hospital mortality has decreased with time (p = 0.08), but the incremental risk of a high hematocrit (p = 0.0003) and of transannular patching (p = 0.05) has persisted. In the current era, the risk of repair in patients with pulmonary stenosis is estimated to be 1.6% (70% confidence limits [CL] 0.7% to 3.5%) at age 5 years, and at age 12 months to be 4.1% (CL 2.7% to 6.3%) without a transannular patch and 7.7% (CL a5.3% to 11%) with one. When pulmonary atresia is present, the probability of hospital death after repair when a valved extracardiac conduit is used is estimated to be lowest (5%; CL 2% to 8%) between 5 1/2 and 16 years of age. No deaths occurred among 53 patients with pulmonary stenosis receiving a primary palliative Blalock-Taussig or Gore-Tex shunt, and six deaths (12%) occurred in 51 patients with pulmonary atresia. Serious interim complications (sudden death, brain abscess) after these shunts occurred in two (1.9%; CL 0.6% to 4.5%) of the patients operated on. No iatrogenic pulmonary arterial problems have been recognized. Protocols based on these results are presented. Many of these selective recommendations may become unnecessary if the damaging effects of cardiopulmonary bypass are overcome by future research.