Ventricle-dependent Coronary Circulation Research Articles

Individualized surgical management of pulmonary atresia with intact ventricular septum

To delineate individualized surgical management strategy for the optimal management of pulmonary atresia with intact ventricular septum (PA/IVS). Between 1997 to 2005, 52 consecutive patients with PA/IVS, 31 male and 21 female, aged 2-9, divided into 2 groups according if right ventricle-dependent coronary circulation (RVDCC) existed, underwent individualized surgical treatment. The patients in Group I (with: RVDCC, n = 6) underwent single ventricle repair. Forty-six patients were in Group II (without RVDCC), 13 of which with severe right ventricular hypoplasia (tricuspid valve Z value < -4)underwent single ventricle repair and 33 of which were with mild to moderate right ventricular hypoplasia (Z value > -4). One critical neonate underwent systemic- pulmonary artery shunt. The other 32 of the 33 patients received right ventricular decompression procedures firstly, and 6 of them presented optimal hemodynamics and oxygenation and achieved biventricular repair. If the patients presented unstable hemodynamics or hypoxemia after right ventricular decompression procedure, an additional shunt was added. 13 young infants (0-2 months) underwent additional systemic-pulmonary artery shunt, and 13 patients (> 3 months-old) underwent additional bidirectional cavopulmonary shunt (1.5 ventricular repair)The patients were followed up for 1-10 years. There were 1 early death in Group I and 7 early deaths in Group II. The total early mortality was 15%. A successful definitive repair was achieved in 26 cases (50%). Follow-up reported 3 late deaths. Individualized surgical management based on the presence of RVDCC, right ventricular hypoplasia grading, right ventricular growth potential, hemodynamic situation, and oxygen saturation after the decompression procedure is helpful to improve the surgical results of PA/IVS

Right ventricle-dependent coronary circulation demonstrated with 64-slice computed tomography

A 12-year-old female patient, who had emergency left modified Blalock–Taussig shunt operation during neonate and a total cavo-pulmonary shunt operation performed at the age of 5 years following a diagnosis of pulmonary atresia with intact ventricular septum (PAIVS), was under regular follow-up. At her last visit, she was admitted with exercised-induced anginal symptoms. …

Pulmonary Atresia With Intact Ventricular Septum: Limitations of Catheter-Based Intervention

Pulmonary atresia with intact ventricular septum (PAIVS) has a wide spectrum of anatomic heterogeneity and invokes a wide variety of treatment strategies. We reviewed the outcome of our patients with PAIVS in order to delineate strategies for the optimal management of PAIVS. In particular, the possibility of avoiding neonatal surgical intervention with catheter-based technology was assessed. The study cohort was composed of all patients presented with PAIVS from January 1999 through December 2005. Demographic and anatomic variables were analyzed to determine association with in-hospital mortality. Forty-four infants with PAIVS underwent catheter valvuloplasty (n = 17) and (or) surgical intervention (n = 42). The mean age and weight of the infants was six days and 3.1 kg, and the average follow-up was 40 +/- 29.5 months. Five (11%) had right ventricle dependent coronary circulation (RVDCC) and six (14%) had Ebstein's anomaly. Five (11%) patients died. Of those who underwent catheter valvotomy, three (18%) underwent shunt placement, 12 (71%) underwent right ventricular outflow tract reconstruction with shunt placement, and only two (12%) did not require a further surgical intervention in the newborn period. Multivariable analyses demonstrated RVDCC (odds ratio 21.3, p = 0.025) and Ebstein's anomaly (odds ratio 16.0, p = 0.038) to be risk factors for in-hospital mortality. Of those patients with Ebstein's anomaly, a single ventricle approach had a better outcome. We demonstrated excellent recent outcomes for patients with PAIVS. Catheter-based interventions rarely avoid surgical repair. The RVDCC and Ebstein's anomaly were associated with high mortality. In patients with Ebstein's anomaly, single ventricular pathway may be the better strategy for this specific patient population.

Radiofrequency pulmonary valvulotomy and valvuloplasty using transductal guidewire rail and translevofemoral venous slide: An unhackneyed solution to a newborn with pulmonary atresia, intact ventricular septum, upstream patent ductus arteriosus, and sigmoid inferior vena cava

Since the pioneer report of transcatheter laser-assisted balloon dilatation in treating patients with pulmonary atresia and intact ventricular septum (PA-IVS) by Qureshi et al. in 1991 [1], radiofrequency pulmonary valvulotomy and valvuloplasty (RFPVV) has superseded the laser-based technique to become the treatment of choice aiming at establishing continuity between the right ventricle and pulmonary trunk, provided that there is a patent infundibulum and absence of a right ventricle-dependent coronary circulation [2].

Pulmonary Atresia, Intact Ventricular Septum, Right Ventricle-Dependent Coronary Artery Circulation, and Systemic Collateral Arteries Supplying the Left Coronary Artery

We present a 14-day-old with pulmonary artery atresia with intact ventricular septum, right ventricle-dependent coronary circulation, a single aortic root coronary ostia (left), congenital collateral arterial supply to the left coronary artery from the left internal mammary artery, and bilateral paravertebral arteries, with obstructive coronary artery lesions.

Does a bidirectional Glenn shunt improve the oxygenation of right ventricle–dependent coronary circulation in pulmonary atresia with intact ventricular septum?

There is a risk of myocardial ischemia in patients with pulmonary atresia and intact ventricular septum associated with the right ventricle-dependent coronary circulation. In this patient group, the oxygen delivery to the myocardium depends on the oxygen saturation of the right ventricular cavity. We hypothesized that bidirectional Glenn shunt would improve the oxygenation of right ventricle-dependent coronary circulation relative to a systemic-pulmonary artery shunt. The reduction of systemic venous return to the right atrium due to a bidirectional Glenn shunt could increase the oxygen saturation of the right ventricle in the clinical setting, when the mixture of systemic and pulmonary venous blood is unchanged at the atrial level. Patients with right ventricle-dependent coronary circulation were defined as those with right ventricle-coronary artery fistulas plus stenoses of the right or left coronary arteries. For 7 patients with right ventricle-dependent coronary circulation before and after bidirectional Glenn shunt, cardiac catheterization was performed and the oxygen saturation of the right ventricular cavity was measured. For all 7 patients, the bidirectional Glenn shunt was performed at a mean age of 18 months. Ischemic changes in the electrocardiogram before the bidirectional Glenn shunt improved after the procedure in 2 patients. The oxygen saturation of the right ventricular cavity before the bidirectional Glenn shunt was 54.6 +/- 8.8%, and that after the BGS significantly increased to 75.6% +/- 5.8% (P < .01). All 7 patients have subsequently undergone the Fontan procedure with excellent results. Early bidirectional Glenn shunt could prevent progression of myocardial ischemia in pulmonary atresia with intact ventricular septum with right ventricle-dependent coronary circulation.

Temporary decompression of the right ventricle to assess the right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum

We repaired a case of pulmonary atresia with intact ventricular septum in which the blood supply to the left anterior descending coronary artery depended on the right ventricle. At the time of a bidirectional Glenn operation, total cardiopulmonary bypass with venous drainage from the right atrium was performed in order to evaluate the safety of right ventricular decompression required for a planned Fontan operation. We confirmed the dependence of the coronary perfusion on the right ventricle by demonstrating transient depression of the ST segment in the epicardial electrocardiogram during temporary decompression of the right ventricle. To prevent ischemic myocardial damage, we then performed an extracardiac Fontan operation with a temporary venous shunt and without cardiopulmonary bypass.

Right Ventricle-dependent Coronary Circulation in Pulmonary Atresia with Intact Ventricular Septum: A Case Report

Pulmonary atresia with intact ventricular septum (PAIVS) is a morphologically heterogeneous lesion and accounts for 1-3% of critically ill infants with congenital heart disease. Numerous surgical approaches have been attempted with varying degrees of success, but the mortality rate is still high in most series. The optimal surgical procedure depends on the size and morphology of the tricuspid valve and right ventricle and the presence or absence of right ventricle-dependent coronary circulation. Therefore, it is pivotal to define the precise morphologic and hemodynamic characteristics, especially coronary artery anatomy. In this report, we describe a full-term female neonate with cyanosis soon after birth. Two-dimensional and color Doppler echocardiography corroborated the diagnosis of PAIVS and showed a small right ventricle. Cardiac catheterization indicated PAIVS and further revealed right ventricle-dependent coronary circulation. A systemic-to-pulmonary artery shunt was constructed with a positive immediate result.

Outcome in infants with pulmonary atresia, intact ventricular septum, and right ventricle–dependent coronary circulation

Management of all patients with pulmonary atresia, intact ventricular septum, and right ventricle-dependent coronary circulation (n = 12) with staged surgery directed toward a Fontan palliation resulted in an 83% 5-year actuarial survival. Both deaths in the study were presumably related to coronary ischemia and occurred in the first 4 months of life.

Veno-venous bypass to prevent myocardial ischemia during right heart bypass operation in PA, IVS, and RV dependent coronary circulation

There is a risk of myocardial ischemia in patients with pulmonary atresia and intact ventricular septum associated with right ventricle dependent coronary circulation, especially during open heart operation. Cardiopulmonary bypass unloads the right ventricle, and thereby reduces the coronary perfusion pressure in an area that is wholly or partly dependent on the right ventricle. We present a veno-venous bypass technique to keep the right ventricle beating and ejecting to supply the oxygenated blood into the right ventricle dependent myocardium and consequently to prevent myocardial ischemia during right heart bypass operation.

Improved results with selective management in pulmonary atresia with intact ventricular septum

Objective: Late outcome of neonatal pulmonary atresia with intact ventricular septum remains poor in most reported series. We have followed a selective approach toward either single ventricle repair versus complete or partial biventricular repair based on the presence of right ventricle–dependent coronary circulation and growth of the right ventricle. Methods: A retrospective chart review was conducted of 47 patients who underwent surgery between January 1991 and September 1998. Results: Sixteen (34%) patients had a right ventricle–dependent coronary circulation, with a tricuspid valve Z-score of –3.0 ± 0.66 versus –2.0 ± 0.95 ( P = .002) for those without a right ventricle–dependent coronary circulation. A systemic–pulmonary artery shunt only was performed in all patients with a right ventricle–dependent coronary circulation, with 1 death. Fourteen of 16 patients with a right ventricle–dependent coronary circulation underwent a bidirectional Glenn shunt at a median of 9 months after their first operation, 9 of whom have had a Fontan procedure (no deaths). In the 31 (66%) patients without a right ventricle–dependent coronary circulation, 6 patients underwent only a systemic–pulmonary artery shunt, 23 had a shunt and right ventricular decompression, and 2 had only a transannular patch. In this group, 10 patients received a 2-ventricle repair, 6 a 1.5-ventricle repair, and 8 patients had a Fontan procedure. There was 1 early death and the overall survival was 98% at 1 year, 5 years, and 7 years. Conclusions: If patients are stratified well, excellent survival can be achieved in the treatment of pulmonary atresia with intact ventricular septum. This result may be at the price of achieving a 1-ventricle as opposed to a 2-ventricle repair. (J Thorac Cardiovasc Surg 1999;118:1046-55)

Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum.

Coronary artery anomalies including 1) right ventricle (RV)-to-coronary artery fistulas, 2) coronary artery stenoses, and 3) coronary occlusions occur in patients with pulmonary atresia with intact ventricular septum (PA-IVS). In some, a large part of the coronary blood supply may depend on the RV. This RV-dependent coronary circulation may determine survival after right ventricular decompression (RVD): RVD may cause RV "steal" in the presence of fistulas alone and ischemia, coronary isolation, or myocardial infarction in the presence of coronary stenoses. Eighty-two patients with PA-IVS who presented between January 1979 and January 1990 were reviewed; 26 (32%) had RV-to-coronary artery fistulas. Of these 26, 23 had adequate preoperative coronary angiograms for analysis. RVD was achieved in 16. Seven of 16 had fistulas only; each survived RVD. Six of 16 had stenosis of a single coronary artery [left anterior descending coronary artery (LAD), four; right coronary artery (RCA), two]; four of six survived RVD. Three of 16 had stenoses and/or occlusion of both the RCA and LAD; all three died shortly after RVD of acute left ventricular dysfunction. 1) Potential RV steal alone does not preclude successful RVD. 2) Fistulas with stenoses to a single coronary artery may not preclude successful RVD. 3) RVD appears to be contraindicated in the presence of stenoses and/or occlusion involving both the right and left coronary systems. Nonsurvival after RVD seems to depend on the amount of the left ventricular myocardium at risk, i.e., that which is distal to coronary artery stenoses, especially when involvement of both coronary arteries limits effective collateralization. Precise definition of coronary arterial anatomy is mandatory in neonates with PA-IVS.

Long-term results in neonates with pulmonary atresia and intact ventricular septum

Out entire institutional experience with pulmonary atresia and intact ventricular septum (1965 through 1987) included 115 patients, 16 of whom died before surgical intervention. Fifty-six percent of surgical patients (n = 99) had angiographic evidence of right ventricle-coronary arterial connections. The early mortality in the surgical group was 27.2%, and the actuarial survival was 24.7% ± 6% at 13 years postoperatively. Multivariate analysis indicated that the presence of ventriculocoronary connections ( p = 0.037), a decreasing ratio between right ventricular and left ventricular pressure at the initial cardiac catheterization ( p = 0.007), and lower weight at operation ( p = 0.001) were incremental risk factors for postoperative death; the presence of Ebstein's anomaly was an additional risk factor in the overall experience (including patients not surgically treated) ( p = 0.01). Nearly all long-term survivors underwent at least one reoperation, including right ventricular outflow tract reconstruction (n = 39) and thromboexclusion of the right ventricle (n = 9). The presence of severe stenosis or interruption of the proximal left anterior descending coronary artery system was a uniformly lethal risk factor for patients undergoing these procedures ( p = 0.0003). We conclude that surgical procedures that successfully decompress the right ventricle will usually result in biventricular circulation in and long-term survival of patients with pulmonary atresia with intact ventricular septum not complicated by Ebstein's anomaly or extensive ventriculocoronary connections. Decompression or thromboexclusion of the right ventricle is contraindicated in patients with ventriculocoronary connections and a right ventricle-dependent coronary circulation.