Criss-cross con concordancia aurícular-ventricular y discordancia ventrículo-arterial: presentación clínica / Criss-cross with atrioventricular concordance and ventriculoarterial discordance: clinical presentation

El corazón con entrecruzamiento de la circulación venosa pulmonar y sistémica a nivel auriculoventricular, denominada criss-cross, puede coexistir en presencia de situs solitus o situs inversus con concordancia o discordancia aurículo-ventricular dependiendo de la rotación cardíaca sobre el eje longitudinal ventricular, a favor o en contra de las manecillas del reloj. Presentamos el caso clínico de un lactante menor masculino con diagnóstico de corazón en criss-cross con concordancia aurículo-ventricular y discordancia ventrículo-arterial que ingresó a nuestro centro con disnea y cianosis. En un primer abordaje se le practica atrioseptostomía quirúrgica, sin embargo, en su evolución intrahospitalaria presenta falla cardíaca global refractaria a tratamiento convencional, por lo cual es llevado a nuevamente a cirugía donde se le realiza intervención de Damus-Kaye-Stansel. En el presente artículo se analizan los detalles de la evaluación ecocardiográfica, el cateterismo cardíaco, la resonancia magnética cardíaca y se plantean las opciones quirúrgicas de esta fascinante entidad patológica.

A heart with cross circulation (criss – cross) can coexist in the presence of situs solitus or situs inversus, with atrioventricular concordance or discordance, depending on the cardiac rotation over the ventricular longitudinal axis, clockwise or clock counterwise. This is the presentation of the clinical case of a male lactant, with diagnosis of criss–cross heart with atrioventricular concordance and ventriculoarterial discordance, who was admitted to these facilities with dyspnoea and cyanosis. In a first approach, a surgical atrioseptectomy was performed on the patient. However, in his intrahospitalary evolution, the patient had a global heart failure refractory to conventional treatment, causing his admission into the operating room for surgery, where the Damus–Kaye–Stensel procedure was performed on him. This article presents an analysis of the details of the echocardiographic evaluation, the cardiac catheterization, and the cardiac magnetic resonance of the clinical case, raising the surgical options for this fascinating pathological entity.

The Clinical Application and Results of Palliative Damus-Kaye-Stansel Procedure / 대한흉부외과학회지

BACKGROUND: The Damus-Kaye-Stansel (DKS) procedure is a proximal MPA-ascending aorta anastomosis used to relieve systemic ventricular outflow tract obstructions (SVOTO) and pulmonary hypertension. The purpose of this study was to review the indications and outcomes of the DKS procedure, including the DKS pathway and semilunar valve function. MATERIAL AND METHOD: A retrospective review of 28 patients who underwent a DKS procedure between May 1994 and April 2006 was performed. The median age at operation was 5.3 months (13 days~38.1 months) and body weight was 5.0 kg (2.9~13.5 kg). Preoperative pressure gradients were 25.3+/-15.7 mmHg (10~60 mmHg). Eighteen patients underwent a preliminary pulmonary artery banding as an initial palliation. Preoperative main diagnoses were double outlet right ventricle in 9 patients, double inlet left ventricle with ventriculoarterial discordance in 6, another functional univentricular heart in 5, Criss-cross heart in 4, complete atrioventricular septal defect in 3, and hypoplastic left heart variant in 1. DKS techniques included end-to-side anastomosis with patch augmentation in 14 patients, classical end-to-side anastomosis in 6, Lamberti method (double-barrel) in 3, and others in 5. The bidirectional cavopulmonary shunt and Fontan procedure were concomitantly performed in 6 and 2 patients, respectively. RESULT: There were 4 hospital deaths (14.3%), and 3 late deaths (12.5%) with a follow-up duration of 62.7+/-38.9 months (3.3~128.1 months). Kaplan-Meier estimated actuarial survival was 71.9%+/-9.3% at 10 years. Multivariate analysis showed right ventricle type single ventricle (hazard ratio=13.960, p=0.004) and the DKS procedure as initial operation (hazard ratio=6.767, p=0.042) as significant mortality risk factors. Four patients underwent staged biventricular repair and 13 received Fontan completion. No SVOTO was detected after the procedure by either cardiac catheterization or echocardiography except in one patient. There was no semiulnar valve regurgitation (>Gr II) or semilunar valve-related reoperation, but one patient (3.6%) who underwent classical end-to-side anastomosis needed reoperation for pulmonary artery stenosis caused by compression of the enlarged DKS pathway. The freedom from reoperation for the DKS pathway and semilunar valve was 87.5% at 10 years after operation. CONCLUSION: The DKS procedure can improve the management of SVOTO, and facilitate the selected patients who are high risk for biventricular repair just after birth to undergo successful staged biventricular repair. Preliminary pulmonary artery banding is a safe and effective procedure that improves the likelihood of successful DKS by decreasing pulmonary vascular resistance. The long-term outcome of the DKS procedure for semilunar valve function, DKS pathway, and relief of SVOTO is satisfactory.

Usefulness and Surgical Strategies of Pulmonary Artery Banding in Functional Univentricular Heart / 대한흉부외과학회지

BACKGROUND: Pulmonary artery banding (PAB)in the functional univentricular heart (UVH)is a palliative procedure for staging toward the Fontan procedure;however,it is known to be a risk factor. MATERIALS AND METHOD: The records of all 37 patients with functional UVHs who underwent surgical palliation using PAB between September 1989 and August 1999 were reviewed retrospectively.We investigated the aortic arch obstruction,the development and progression of subaortic stenosis after PAB,and risk factor of mortality according to surgical method. RESULT: In 37 neonates and infants with single ventricular physiology,aortic arch obstruction was combined in 7.There were 6 early deaths (16.2%)after PAB and 3 late deaths (8.1%)after Fontan operation.The actuarial overall survival including early mortality at 3 and 5 years were 8 0 .7+/-6.6%,72.2 +/-8.2% respectively. Among 31 patients who survived PAB,27 patients (87.1%)could become candidates for Fontan operation;22 patients(71.0%)completed Fontan operation with 3 deaths and 5 were waiting bidirectional cavopulmonary shunt(BCPS)or Fontan operation (follow-up mean 4.5 year,minimal 2 year). Subaortic stenosis developed in 8 patients after PAB (8/29,27.6%);3 cases in the patients without arch anomaly (3/22,13.6%)and 5 in those with arch anomal y (5/7,71.4%).The subaortic stenosis was managed with Damus-Kaye-Stansel procedure (DKS)in 6 patients without operative mortality and conal septum resection in 2 without long-term survivor. Analysis of risk factors established that aortic arch obstruction was strongly associated with subaortic stenosis (p<0.001).The only risk factor of late mortality was Fontan procedure without staged palliation by BCPS (p=0.001). CONCLUSION: PAB is effective as an initial palliative step in functional UVH.And the high risk group of patients with aortic obstruction can undergo effective short-term PAB as an initial palliative step,with subsequent DKS for subaortic stenosis.This strategy,initial PAB and careful surveillance,and early relief of subaortic stenosis can maintain acceptable anatomy and hemodynamics for later Fontan procedures.