Diagnóstico prenatal de enfermedad de canal atrio-ventricular, análisis epidemiológico y experiencia CERPO entre 2003 y 2021 / Prenatal diagnosis of atrio-ventricular canal disease, epidemiological analysis and CERPO experience between 2003 and 2021

Antecedentes: En Chile, la mitad de los casos de mortalidad perinatal son atribuibles a anomalías congénitas, y un tercio de estas corresponde a cardiopatías congénitas. Aproximadamente un 35% de estos últimos requerirán cirugía antes del año de vida, por lo que la pesquisa prenatal impacta profundamente en el pronóstico. Objetivo: Dar a conocer los resultados perinatales de pacientes con diagnóstico prenatal de canal atrio-ventricular controlados en el Centro de Referencia Perinatal Oriente (CERPO) entre los años 2003 y 2021, su asociación a otras anomalías, características demográficas y pronóstico a un año. Métodos: En este estudio se puede apreciar que tanto el pronóstico como el plan terapéutico posnatal dependerán de la presencia de otras alteraciones morfológicas y del estudio genético. De los factores estudiados, se puede concluir que tanto la presencia de anomalías cardiacas asociadas, como el grado de insuficiencia valvular y el tipo de canal no son predictores de la sobrevida perinatal. Conclusiones: Finalmente, en relación con la sobrevida posnatal, en este estudio, la sobrevida a un año de los recién nacidos vivos fue de un 52%, pero al desglosarlo en los niños con cariotipo euploide y trisomía 21, estos valores se tornan muy distintos, 44 y 81% respectivamente.

Background: In Chile, half of the perinatal mortality cases are attributable to congenital anomalies, and one third of these correspond to congenital heart disease. Approximately 35% of the later will require surgery before one year of life, so prenatal screening has a profound impact on the prognosis. Objective: To present the perinatal results of patients with a prenatal diagnosis of atrio-ventricular canal controlled at Centro de Referencia Perinatal Oriente CERPO) between 2003 and 2021, its association with other anomalies, demographic characteristics, and 1-year prognosis. Methods: In this study it can be seen that both the prognosis and the postnatal therapeutic plan will depend on the presence of other morphological alterations and the genetic study. From the factors studied, it can be concluded that the presence of associated cardiac anomalies, the degree of valvular insufficiency, and the type of canal are not predictors of perinatal survival. Conclusions: Finally, in relation to postnatal survival, in this study, the 1-year survival of live newborns was 52%, but when broken down into children with euploid karyotype and trisomy 21, these values become very different, 44 and 81% respectively.

Differences in morbidity and mortality in Down syndrome are related to the type of congenital heart defect.

Morbidity and mortality in Down syndrome (DS) are mainly related to congenital heart defects (CHDs). While CHDs with high prevalence in DS (typical CHDs), such as endocardial cushion defects, have been extensively described, little is known about the impact of less common CHDs (atypical CHDs), such as aortic coarctation and univentricular hearts. In our single-center study, we analyzed, in observational, retrospective manner, data regarding cardiac features, surgical management, and outcomes of a cohort of DS patients. Literature review was performed to investigate previously reported studies on atypical CHDs in DS. Patients with CHDs were subclassified as having typical or atypical CHDs. Statistical analysis was performed for comparison between the groups. The study population encompassed 859 DS patients, 72.2% with CHDs, of which 4.7% were atypical. Statistical analysis showed a significant excess in multiple surgeries, all-cause mortality and cardiac mortality in patients with atypical CHDs (p = .0067, p = .0038, p = .0001, respectively). According to the Kaplan-Meier method, survival at 10 and 40 years was significantly higher in typical CHDs (99 and 98% vs. 91 and 84%, log rank <0.05). Among atypical CHDs, it seems that particularly multiple complex defects in univentricular physiology associate with a worse outcome. This may be due to the surgical difficulty in managing univentricular hearts with multiple defects concurring to the clinical picture or to the severity of associated defects themselves. Further studies need to address this specific issue, also considering the higher pulmonary pressures, infective complications, and potential comorbidities in DS patients.

Parámetros clínicos, ecocardiográficos y analíticos en pacientes con defecto del septo auriculoventricular con y sin síndrome de Down / Clinical, echocardiographic and analytical parameters in patients with atrioventricular septal defect with and without Down syndrome

No disponible

Cardiovascular magnetic resonance parameters associated with early transplant-free survival in children with small left hearts following conversion from a univentricular to biventricular circulation.

BACKGROUND: We sought to identify cardiovascular magnetic resonance (CMR) parameters associated with successful univentricular to biventricular conversion in patients with small left hearts. METHODS: Patients with small left heart structures and a univentricular circulation who underwent CMR prior to biventricular conversion were retrospectively identified and divided into 2 anatomic groups: 1) borderline hypoplastic left heart structures (BHLHS), and 2) right-dominant atrioventricular canal (RDAVC). The primary outcome variable was transplant-free survival with a biventricular circulation. RESULTS: In the BHLHS group (n = 22), 16 patients (73%) survived with a biventricular circulation over a median follow-up of 40 months (4-84). Survival was associated with a larger CMR left ventricular (LV) end-diastolic volume (EDV) (p = 0.001), higher LV-to-right ventricle (RV) stroke volume ratio (p < 0.001), and higher mitral-to-tricuspid inflow ratio (p = 0.04). For predicting biventricular survival, the addition of CMR threshold values to echocardiographic LV EDV improved sensitivity from 75% to 93% while maintaining specificity at 100%. In the RDAVC group (n = 10), 9 patients (90%) survived with a biventricular circulation over a median follow-up of 29 months (3-51). The minimum CMR values were a LV EDV of 22 ml/m² and a LV-to-RV stroke volume ratio of 0.19. CONCLUSIONS: In BHLHS patients, a larger LV EDV, LV-to-RV stroke volume ratio, and mitral-to-tricuspid inflow ratio were associated with successful biventricular conversion. The addition of CMR parameters to echocardiographic measurements improved the sensitivity for predicting successful conversion. In RDAVC patients, the high success rate precluded discriminant analysis, but a range of CMR parameters permitting biventricular conversion were identified.

Echocardiographic features defining right dominant unbalanced atrioventricular septal defect: a multi-institutional Congenital Heart Surgeons' Society study.

BACKGROUND: Definition and management of right dominant unbalanced atrioventricular septal defect (AVSD) remains challenging because unbalance entails a spectrum of left heart hypoplasia. Previous work has highlighted atrioventricular valve (AVV) index as a reasonable defining echocardiographic measure. We sought to assess which additional echocardiographic features might provide further characterization. METHODS AND RESULTS: From a multi-institutional cohort of complete AVSD, 52 preoperative echocardiograms of patients with presumed right dominant unbalanced AVSD (based on AVV index) and 60 randomly selected preoperative echocardiograms from patients with presumed balanced AVSD were reviewed. Cluster analysis of echocardiographic variables was used to group patients with similar features. Discriminant function analysis was used to explore which variables differentiated these groups. Three groups were identified from the cluster analysis. Echocardiographic variables that differentiated these groups were right ventricle:left ventricle inflow angle, LV width/LV length, left AVV color diameter at smallest inflow, left AVV color diameter at annulus, right AVV overriding left atrium, and LV width. Based on procedures and outcomes, 1 group likely represented balanced patients, whereas 2 groups with similar outcomes likely represented unbalanced patients. The dominant differentiating echocardiographic variable between the 3 cluster groups was the right ventricle:LV inflow angle (partial R²=0.86), defined as the angle between the base of the right ventricle and LV free wall, using the crest of the ventricular septum as apex of the angle. CONCLUSIONS: The angle of right ventricle/LV inflow and other surrogates of inflow may be important defining echocardiographic measures of right dominant unbalanced AVSD, although confirmation is needed.

Biventricular conversion after single-ventricle palliation in unbalanced atrioventricular canal defects.

BACKGROUND: Management of unbalanced common atrioventricular canal (UCAVC) defect by a single-ventricle (SV) approach frequently results in poor outcomes, especially in trisomy 21 patients. In this report we describe our results with conversion to biventricular circulation in UCAVC patients with SV palliation. METHODS: Retrospective review of patients with UCAVC undergoing biventricular conversion from prior SV palliation between 2003 and 2011 was conducted. Mortality and freedom from reinterventions were analyzed using nonparametric methods. RESULTS: Sixteen children with UCAVC (8 patients [50%] were left dominant) and prior SV palliation underwent conversion to biventricular circulation between 2003 and 2011. Median follow-up was 18 months (range, 3 to 94 months). Surgical indications included worsening cyanosis, severe atrioventricular valve regurgitation, or failing bidirectional Glenn or Fontan physiology. All patients had either unequal distribution of the common atrioventricular valve of greater than 60% or one hypoplastic ventricle. By magnetic resonance imaging or computed tomography, 8 patients with right dominant atrioventricular canal had a median left ventricular end-diastolic volume of 32 mL/m(2) (range, 22 to 35 mL/m(2)). Eight patients with a left dominant atrioventricular canal had a median right ventricular end-diastolic volume of 42 mL/m(2) (range, 26 to 64 mL/m(2)). Eleven patients (69%) had trisomy 21, and 3 patients (19%) had heterotaxy. Stages of palliation included stage I in 2 patients, bidirectional Glenn in 10 patients, hemi-Fontan in 2 patients, and Fontan in 2 patients. There was 1 (6%) operative (right ventricle dominant) and 1 (6%) late death (left ventricle dominant). Eight patients required reinterventions, 3 (19%) surgical and 6 (38%) catheter-based. On follow-up, all had improvement in cyanosis and symptoms. CONCLUSIONS: Biventricular conversion from failing SV palliation in UCAVC can be accomplished with an acceptable early and late morbidity and mortality, although need for reintervention was not uncommon.

Results of surgical repair of atrioventricular septal defect with double-orifice left atrioventricular valve.

OBJECTIVE: The outcome of surgical correction of atrioventricular septal defect with double-orifice left atrioventricular valve has improved in recent years but is still reported to be associated with high mortality and reoperation rates. Controversy exists about the management of the accessory orifice. We evaluated our results with correction of atrioventricular septal defect with double-orifice left atrioventricular valve. METHODS: Between 1975 and 2006, 21 patients underwent correction of atrioventricular septal defect with double-orifice left atrioventricular valve. Clinical data were obtained by means of retrospectively reviewing inpatient and outpatient medical records. To evaluate the influence of double-orifice left atrioventricular valve on mortality and the need for reoperation, a comparison was made with 291 consecutive patients who, during the same period, underwent correction of atrioventricular septal defect without double-orifice left atrioventricular valve. RESULTS: None of the 21 patients with double-orifice left atrioventricular valve had undergone a previous operation. The accessory orifice was managed with different techniques depending on the severity of the regurgitation. There was no in-hospital mortality, and there were 3 late deaths. Seven patients required 12 reoperations, 7 for left atrioventricular valve insufficiency. Double-orifice left atrioventricular valve had no influence on mortality but was a significant predictor for reoperation compared with repair of atrioventricular septal defect without double-orifice left atrioventricular valve. At the latest follow-up, all 18 survivors were in New York Heart Association functional class capital I, Ukrainian without medication. Only 1 patient showed residual mild left atrioventricular valve insufficiency. CONCLUSION: Atrioventricular septal defect with double-orifice left atrioventricular valve can be repaired with low mortality. However, double-orifice left atrioventricular valve is a predictor for reoperation. The accessory orifice is often competent and should then be left untouched. If regurgitation of the accessory orifice is present, this is best managed with suture or patch closure.

[The ostium primum or partial atrioventricular septal defect]. / La CIA, de type ostium primum ou canal atrio-ventriculaire partiel.

Often assimilated to simple inter-atrial communication, the ostium primum, or partial atrio-ventricular septal defect, is an entity that is characterized by a different embryological mechanism and requires some specific surgical expertise. Basically, knowledge of the morphology of the common atrioventricular valve with 5 components, the topography of the A-V node and His bundle, and the ventricular consequences of the absence of atrio-ventricular septal structures must be taken into account.

Patología coronaria no arteriosclerótica como causa de muerte súbita en adultos. Casuística del Instituto de Medicina Legal de Valencia (1997-2005) / Nonatherosclerotic coronary artery disease presenting as sudden death in adults. Casuistic from the Institute of Legal Medicine of Valencia (Spain) (1997-2005)

Introducción y objetivos. En los países industrializados, la causa más frecuente de muerte súbita cardíaca (MSC) en el adulto es la isquemia miocárdica aguda por arterioesclerosis coronaria. Sin embargo, existen otras patologías coronarias, congénitas o adquiridas, que pueden producir isquemia miocárdica y muerte súbita: origen anómalo, hipoplasia, estenosis del ostium, aneurisma, disección, localización alta del ostium, puente miocárdico, vasculitis. El objetivo del presente trabajo es revisar la casuística de este tipo de patología coronaria no arterioesclerótica en las 8003 autopsias realizadas en el Instituto de Medicina Legal de Valencia (IMLV) entre los años 1997-2005. Método: Los criterios de inclusión de los casos para este estudio han sido: edad superior a 14 años, patología coronaria no arterioesclerótica como causa de muerte y circunstancias de la muerte compatibles con la definición de muerte súbita. Resultados: Se han encontrado 9 casos de muerte súbita en adultos con patología coronaria no arterioesclerótica como causa de muerte: 2 casos de puente miocárdico, 6 casos de disección coronaria y 1 caso de origen anómalo de coronaria. Conclusiones: La patología coronaria no arterioesclerótica, aunque es poco frecuente como causa de MSC, debemos conocerla y poder diagnosticarla macroscópicamente en la sala de autopsias. Siempre debe descartarse otra posible causa de muerte mediante los estudios complementarios químico-toxicológicos e histopatológicos. Es importante conseguir la máxima información clínica anterior al fallecimiento que pueda ayudar al diagnóstico en vida de este tipo de patologías, ya que en la mayoría de los casos sigue siendo un hallazgo postmortem

Introduction and objectives. Coronary atherosclerosis is the most frequent cause of acute myocardial ischemia and sudden cardiac death in industrialized countries. However, there are others diseases, congenital or acquired, that can produce myocardial ischemia and sudden death: anomalous origin, hypoplasia, ostial stenosis, aneurysms, dissections, high take-off, myocardial bridges, vasculitis. The aim of this paper is to review the frequency and characteristics of nonatherosclerotic coronary artery disease in 8003 forensic autopsies performed at the Institute of Legal Medicine of Valencia (Spain) between 1997-2005. Method: The criteria for the case selection have been: - subjects over 14 years old, sudden death caused by nonatherosclerotic coronary artery disease and circumstances of death compatible with the definition of sudden death. Results. Nine cases of sudden cardiac death have been found in adults of which two (2) cases of myocardial bridges, six (6) cases of coronary dissection and one (1) case of anomalous origin. Conclusions. Although non-atherosclerotic coronary artery disease is a rare cause of sudden cardiac death, we must be aware of it and be able to diagnose it in the autopsy-room. Other possible causes of death should always be eliminated through additional complementary chemo-toxicological- and histological analysis. It is important to obtain the maximum amount of clinical information, which will assist the diagnosis of this type of pathology, since in most cases it continues to be a post-mortem finding

Cleft mitral valve without ostium primum defect: anatomic data and surgical considerations based on 41 cases.

BACKGROUND: Cleft mitral valve without an ostium primum defect, referred to as isolated cleft mitral valve, has been the subject of many reports; yet its morphology and operability remain incompletely understood. METHODS: The anatomic findings in 36 postmortem cases, five explanted hearts, and relevant clinical data constitute the material basis of this report. Cardiac catheterization data were available in 29 cases and two-dimensional echocardiograms in 13 cases. RESULTS: Twenty cases had normally related great arteries with subpulmonary conus. Of these cases 4 (20%) had tetralogy of Fallot and 1 had tricuspid atresia. Twenty-one cases had abnormal ventriculoarterial relationships with subaortic or bilateral conus resulting in transposition in 16 (76%) and double-outlet right ventricle in 5 (24%). In the cases with normally related great arteries, the morphology of the ventricular septal defect and the mitral cleft were similar to those of the more complete forms of atrioventricular canal defects. The mitral cleft usually resulted in progressive mitral regurgitation, which can be treated by surgical closure of the cleft. In the cases with abnormal conus, the morphology of the ventricular septal defect and the mitral cleft did not resemble atrioventricular canal defects. The attachment of the cleft usually produced obstruction of the left ventricular pulmonary outflow tract. Surgical repair of the cleft cannot eliminate this obstruction. CONCLUSIONS: There are two different anatomic types of isolated cleft mitral valve: the canal type, and abnormal conus type. Diagnosis of the associated ventriculoarterial relationships helps to guide their surgical treatment.

Embolismo poplíteo bilateral secundario a infección fúngica sobre cicatriz de aortotomía / Bilateral popliteal embolism secondary to the fungal infection of the scar tissue resulting from an aortotomy

Introducción. La infección de una cicatriz de aortotomía es una entidad clínica muy poco frecuente, más aún si es de origen fúngico. Caso clínico. Varón de 66 años que acudió a urgencias por fiebre, hipotensión e isquemia aguda de ambos miembros inferiores, de 24 horas de evolución. Entre sus antecedentes patológicos destacaba la sustitución valvular aórtica hacía 17 años por una válvula mecánica. La arteriografía diagnóstica mostró la oclusión embólica bilateral de la poplítea distal, y se le practicó una tromboembolectomía poplítea bilateral con remisión del síndrome isquémico agudo. El análisis anatomopatológico de los émbolos mostró hifas compatibles con Aspergillus sp. Se inició el tratamiento con anfotericina B endovenosa y sustitución de la válvula. En el acto operatorio, se detectó la presencia de una vegetación sobre la aortotomía previa. En el postoperatorio de la cirugía cardíaca el paciente falleció por un infarto agudo de miocardio. Conclusiones. Se presenta una forma infrecuente de aortitis, que lleva implícitas dificultades diagnósticas y terapéuticas, y que habitualmente se acompaña de altas tasas de mortalidad (AU)

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.

Direct closure of ostium primum defect in the repair of atrioventricular septal defect.

BACKGROUND: Patch closure is generally performed for atrial septation of an atrioventricular septal defect. We recently developed a new surgical technique for repairing atrioventricular septal defects that avoids the use of any patch material for closing the atrial septal defect. We report our experience with this procedure. METHODS: Seven patients (complete type: 5, partial type: 2) underwent this new operation. The diameters of the atrial septal defects were measured by transesophageal echocardiography. The preoperative electrocardiograms were compared with those taken after the operations. RESULTS: Diameters of the atrial defects ranged from 3 to 10 mm. Electrocardiograms before and after the operations did not change. No significant atrioventricular valve regurgitation and no residual shunts were detected by postoperative echocardiography. CONCLUSIONS: This method simplifies the repair of atrioventricular septal defects. In the short-term results, no arrhythmia and no valve regurgitation was seen.

Twenty-year experience with repair of complete atrioventricular septal defects.

BACKGROUND: To determine factors predicting mortality and morbidity after repair of complete atrioventricular septal defect, we retrospectively analyzed preoperative, operative, and postrepair factors on the outcome of 115 consecutive complete atrioventricular septal defect repairs at The Children's Hospital of Wisconsin between January 1974 and December 1993. METHODS: For the entire experience the operative mortality was 13.9% (16 patients). During the most recent era, January 1988 to December 1993, operative mortality was 3.6% (2 of 55 patients). This was significantly improved from the two previous eras, January 1974 to December 1980, 28% (7 of 25) and January 1981 to December 1987, 20% (7 of 35 patients) (p = 0.02). There were seven late deaths; 10-year actuarial survival, including operative mortality was 81%. Age at complete repair decreased; before 1982 all patients were more than 12 months of age, whereas after 1982 64% (56 of 88 patients) were 12 months of age or less. RESULTS: Moderate or severe preoperative left atrioventricular valve regurgitation was not a risk factor for operative mortality. For operative survivors with moderate to severe preoperative left atrioventricular valve regurgitation (n = 17), late postoperative left atrioventricular valve regurgitation (follow-up data available on 15 patients) was significantly reduced (severe = 1, moderate = 5, mild = 9; p = 0.007). CONCLUSIONS: Early mortality was predicted by the era of surgical repair. Conversion to routine repair during infancy was achieved with a simultaneous decrease in operative mortality. For patients with moderate to severe preoperative left atrioventricular valve regurgitation, significant improvement in the degree of left atrioventricular valve regurgitation can be expected without an increase in operative or late mortality or morbidity.

Partial atrioventricular canal with congestive heart failure in the first year of life: surgical options.

BACKGROUND: An important subgroup of patients with partial atrioventricular canal require an operation in the first year of life because of refractory congestive heart failure. METHODS: From June 1982 to April 1995, of 128 patients with partial atrioventricular canal, 35 patients (27%) underwent surgical treatment at less than 1 year of life. Associated cardiac anomalies were present in 22 patients. Only 7 patients (20%) had Down's syndrome. Five patients with left ventricular hypoplasia underwent aortic coarctectomy (3 patients) or Norwood operation (2 patients). The other 30 patients underwent anatomic repair in 24 cases and aortic coarctectomy in 6. The surgical results of patients submitted for anatomic repair were retrospectively correlated with the echocardiographic mitral valve diameter. RESULTS: There were 7 deaths (29%) after anatomic repair, 2 (22%) after aortic coarctectomy, and 2 (100%) after Norwood operation. Infants with a mitral valve diameter less than 2.5 x 10-2 m/m2 died at repair. In a mean follow-up of 73.5 months there were five secondary mitral valve plasties and three repairs after aortic coarctectomy. CONCLUSIONS: Among patients with partial atrioventricular canal, there is an important subgroup with clinical signs of heart failure in the first year of life. Left-sided obstructive lesions and complex mitral valve anomalies seem to play a fundamental role in the clinical evolution and prognosis of these patients. The echocardiographic mitral valve diameter may be useful for determining the correct surgical indication.

Long-term follow-up after surgical repair of ostium primum atrial septal defects in adults.

OBJECTIVES: We sought to determine the long-term survival rates and defect-related morbidity of adult patients who undergo surgical repair of an ostium primum atrial septal defect. BACKGROUND: The natural history of patients undergoing such surgical repair in adulthood remains unclear. METHODS: We followed up 33 patients who underwent surgical correction of an ostium primum atrial septal defect at our institution at a mean age of 42 years (range 20 to 73); 12 of these patients were > 50 years old at the time of operation. Four patients had moderate preoperative exercise incapacity (New York Heart Association functional class > II) and six had atrial fibrillation. Nine and four patients, respectively, had a preoperative mean pulmonary artery pressure > 25 mm Hg or pulmonary vascular resistance > 4 Wood U. Autologous pericardium was used to patch the defect in 30 patients (91%). Mitral valvuloplasty, consisting of cleft repair (n = 10), and mitral valve replacement (n = 2) were performed selectively. RESULTS: There were no operative deaths. At a mean follow-up interval of 5.3 years (range 1 to 18.2), all 28 surviving patients are free of exercise limitation (functional class 1). Late postoperative deaths occurred in five patients (15%) and were related to myocardial infarction, stroke, hepatic failure, renal failure or sepsis. Reoperation within the 1st postoperative year was required in two patients (6%) because of a residual ostium primum defect in one and severe mitral regurgitation in the other. The presence of advanced age at operation, symptoms, atrial arrhythmias, mitral regurgitation or moderately increased pulmonary vascular resistance did not predict late postoperative mortality, complications or functional capacity. CONCLUSIONS: An ostium primum defect can be repaired in adult patients with the expectation of excellent long-term results, independent of age at operation and preoperative mitral valve function and despite the presence of atrial fibrillation or moderately elevated pulmonary vascular resistance.

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

OBJECTIVE: 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. METHODS: 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. RESULTS: 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%). CONCLUSIONS: 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.

Pulmonary vascular disease and operative indications in complete atrioventricular canal defect in early infancy.

Pulmonary vascular disease was morphometrically analyzed in 67 patients (mean age, 19 months) with isolated complete atrioventricular canal defect. Complete obstruction of the small pulmonary arterial lumen resulting from acute fibrous proliferation and atrophy of the peripheral arterial media, which were considered absolute operative contraindications, were characteristic in six patients with Down's syndrome. Morphometric analysis of medial thickness revealed that thinning of the media of the small pulmonary arteries is generally observed at around 6 months of age in patients with complete atrioventricular canal defect and that the media in patients who have complete atrioventricular canal defect and Down's syndrome was thinner than that in such patients without Down's syndrome. These results suggest that thinning of the media as a result of two factors--Down's syndrome and aging--facilitates the rapid occurrence of fibrous intimal proliferation. Therefore intracardiac repair is desirable within 6 months of life, before medial thinning, in patients with complete atrioventricular canal defect and Down's syndrome. Excluding patients with absolute operative contraindications, the scores of the index of pulmonary vascular disease in operative survivors were below 2.0 and death occurred when scores were more than 2.2. The pulmonary vascular resistances measured in room air and by the oxygen inhalation and tolazoline tests in patients with operative contraindications were more than 7.3, 3.8, and 6.6 units.m2, respectively. We thus conclude that lung biopsy should be undertaken for patients in whom pulmonary vascular resistance is beyond these values to determine the appropriateness of surgical intervention.

Surgical repair of complete atrioventricular canal defects in infancy. Twenty-year trends.

Case histories of 301 patients with complete atrioventricular canal defect presenting to our institution in infancy between January 1972 and January 1992 were reviewed with the purpose of identifying the factors responsible for the observed improvement in perioperative mortality over this time period. A retrospective analysis of hospital records examined 46 patient-related, morphologic, procedure-related, and postoperative variables for associations with perioperative death and reoperation. Operative mortality decreased significantly over the period of the study from 25% before 1976 to 3% after 1987 (p < 0.0001). A number of the 46 variables examined showed trends over time that were similar to that for mortality. Palliative procedures decreased over time. Reoperation for most residual lesions also decreased to the degree that they were essentially eliminated in recent years. The exception to this was reoperation for postoperative left atrioventricular valve regurgitation, which also decreased but remained at 7% in recent years. Both technical and support-related procedural variables showed no trends over time, with the exception of the performance of left atrioventricular valve annuloplasty, which increased over time. Closure of the left-sided cleft was performed in 61% of the patients, with no trend over time. Annuloplasty and cleft closure were not associated with less postoperative left atrioventricular valve regurgitation, fewer reoperations, or lower mortality. Multivariate logistic regression analysis identified only earlier year of operation, the presence of double-orifice left atrioventricular valve, and postoperative residual regurgitation of the left atrioventricular valve as risk factors for death. Experience-related improvements in technical precision achieved over time best account for the reduction in the rate of reoperation for most types of residual lesions and also for the reduction in mortality. The only residual lesion that has not been essentially completely eliminated is left atrioventricular valve regurgitation, with reoperation for this lesion having been reduced in recent years, but not eliminated. Improved understanding of the structural and functional variability of the atrioventricular valve in this lesion may be necessary before postoperative dysfunction of this valve can be completely eliminated.

Atrioventricular canal defect: two-patch repair and tricuspidization of the mitral valve.

Between November 1978 and May 1991, a two-patch repair with tricuspidization of the left atrioventricular valve was performed in 104 patients with complete atrioventricular canal defect. Mean age was 12 months for the 68 patients who underwent primary repair and 22 months for the 36 patients who had previous pulmonary artery banding. Eleven patients died within 30 days of operation and 4 died late. Reoperation was carried out in 2 patients. Eighty-six patients were in sinus rhythm after operation. One patient required a permanent pacemaker for complete heart block. Follow-up data for 1 to 13 years are available in 80 of 89 survivors (90%). Actuarial survival at 13 years is 81% and event-free survival is 92%. Seventy-six patients are in New York Heart Association class I and 4 in class II. Postoperative echocardiogram of these patients showed minimal or no mitral regurgitation. The advantages of this technique are preservation of valve integrity, durable mitral valve repair, and rare reoperation or heart block even in younger patients.