Diminutive Pulmonary Arteries Research Articles

Ventricular Septal Defect Patch Fenestration in Repair of Tetralogy of Fallot With Diminutive Pulmonary Arteries

Ventricular Septal Defect Patch Fenestration in Repair of Tetralogy of Fallot With Diminutive Pulmonary Arteries

Ventricular septal defect patch fenestration in repair of tetralogy of fallot with diminutive pulmonary arteries

Ventricular septal defect patch fenestration in repair of tetralogy of fallot with diminutive pulmonary arteries

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

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

The management of tetralogy of fallot with pulmonary atresia and diminutive pulmonary arteries

Since September 1991, 14 consecutive patients with tetralogy of Fallot, pulmonary atresia, and diminutive pulmonary arteries have undergone staged repair. All patients had multiple aortopulmonary collateral arteries and the ductus arteriosus was absent in 11. Mean sizes of the right and left pulmonary arteries were 2.2 ±0.7 mm and 1.9 ±0.8 mm, respectively (range 0.5 to 3.0 mm). Eight patients (57%) have subsequently received complete repair. Age at initial procedure (shunt, right ventricle–pulmonary artery conduit, or direct aorta–pulmonary artery anastomosis) in this group was 5.3 ±6.8 months. The number of operative procedures to achieve complete repair was 2.9 ±0.8 per patient (range 2 to 4). Intraoperative postrepair peak right ventricle–left ventricle pressure ratio was 0.57 ±0.17. Six of 8 patients (75%) required additional interventional procedures (mean 1.5 ±1.2 per patient) for angioplasty of peripheral pulmonary artery stenoses, coil embolization of aortopulmonary collateral arteries, or intraoperative insertion of intravascular pulmonary artery stents. Mean follow-up from complete repair was 8.7 ±8.3 months (range 0.5 to 23.8 months) and is complete. There was one in-hospital death at 45 days, and one late cardiac death at 20.3 months. Six patients had initial palliative operations (unifocalization, right ventricle–pulmonary artery conduit, direct aorta–pulmonary artery anastomosis, or transannular outflow patch) but have not undergone complete repair. Age at initial procedure in this group was 27.9 ± 56.9 months (range 0.27 to 155 months), and mean follow-up from initial procedure was 10.9 ± 11.2 months (range 0 to 31.4 months). The operative mortality rate was 33% (2 of 6 patients). There was one late noncardiac death at 5.3 months. Three patients are awaiting further intervention or repair. This experience suggests that complete repair is feasible even in patients with extremely diminutive pulmonary arteries (≤3.0 mm). Pulmonary artery growth is facilitated by early (3 to 6 month) establishment of central pulmonary artery flow by right ventricle–pulmonary artery conduit (pulmonary arteries >1.5 mm) or by direct ascending aorta–pulmonary artery anastomosis (pulmonary arteries <1.5 mm). Subsequent interventional catheterization and operative procedures as required for pulmonary artery stenoses and coil embolization of collateral arteries allow continued recruitment of central pulmonary arteries and may obviate or minimize the need for unifocalization procedures. (J THORAC CARDIOVASC SURG 1995;110:1521-33)

Long-term palliation after systemic-to-pulmonary artery shunt using an internal mammary artery

We report the long-term results after systemic-to-pulmonary artery shunts using the internal mammary arteries in 2 patients. The systemic-to-pulmonary artery shunts using the internal mammary arteries were created in 2 children. Both of them are doing well 11 years and 7 years after operation. This procedure could be a reasonable alternative to classic Blalock-Taussig shunt for patients with diminutive pulmonary arteries as it offered good long-term palliation.

Creation of a Systemic-to-Pulmonary Artery Shunt By Use of an Internal Mammary Artery

The creation of a systemic-to-pulmonary artery shunt by use of the internal mammary artery (IMA) in a 2-year-old boy with complex congenital heart defects and diminutive pulmonary arteries is described. This procedure may offer advantages over standard methods in selected patients with extremely small pulmonary arteries. The pliability, favorable anatomical position, and growth capability of the IMA reduce chances for complications, such as stenosing, kinking, and stretching of the pulmonary artery, which can cause it to become distorted, making further definitive repair difficult.

Tetralogy of fallot, pulmonary atresia, and diminutive pulmonary arteries

The surgical management of classical tetralogy of Fallot (TOF), with right ventricular-to-pulmonary artery continuity and pulmonic stenosis, has been well established and standardized, and both early and late results are good, even in critically ill infants. In other patients with pulmonary atresia with ventricular septal defect, however, significant additional therapeutic challenges are seen, primarily because of the wide range of origin, size, and distribution of pulmonary blood supply found in this entity. The most fortunate subset of patients with tetralogy of Fallot with pulmonary atresia (TOFPA) are those with valvar pulmonary atresia, a ductus-dependent pulmonary arterial circulation and essentially normal pulmonary arteries. At the other end of anatomic complexity are those patients with TOF-PA without a patent ductus arteriosus (PDA) and with diminutive or absent central pulmonary arteries. In these patients pulmonary blood flow is supplied exclusively by aortopulmonary collateral arteries (APCAs). Because of the significant variations in pulmonary arterial structure and blood flow, no single effective treatment plan is applicable to this diverse group of patients. Generally,

Right pulmonary artery development after modified Blalock-Taussig shunt (MBTS) in infants with pulmonary atresia, VSD and confluent pulmonary arteries

The ideal palliation for infants with pulmonary atresia, ventricular septal defect and confluent pulmonary arteries should promote normal development of the pulmonary artery branches. In 26 patients who survived a modified Blalock-Taussig shunt (MBTS) in the first year of life, the right pulmonary artery was measured before and after operation by two dimensional (2D) echocardiography. In each patient its size was compared to the normal value for the same body surface area. The patients were divided according to this ratio and according to the real size (mm). Although increased in all cases but two, the size of the right pulmonary artery remained remarkably less than normal in patients with an initially small right pulmonary artery. In view of these results, early palliative enlargement of the right ventricular outflow tract is probably advisable in infants with diminutive pulmonary arteries who do not show adequate pulmonary branch development after MBTS.

Diminutive pulmonary artery growth following right ventricular outflow tract enlargement

We have assessed the clinical results and angiographic pulmonary artery growth following construction of the right ventricular outflow tract in 10 children with tetralogy of Fallot or pulmonary atresia. All cases were associated with diminutive pulmonary arteries and considered unsuitable for total corrective surgery. The mean age of the children was 34 months, mean weight 10 kg and mean body surface area 0.48 m 2. The right ventricular outflow tract was constructed by insertion of a patch of dura mater (5 patients), pericardium (2 patients), homograft valved conduit (2 patients) and infundibulectomy (1 patient). The ventricular septal defect was not closed. Study of the patients between 13 and 37 months postoperatively revealed an increase in mean arterial oxygen saturation of 22% ( P < 0.01) and a fall in mean haemoglobin concentration of 1.6 g% ( P < 0.05). Overall change in pulmonary artery diameter compared to that of the trachea was insignificant although in individual cases a 50% increase in diameter was shown. Palliation of symptoms was equivalent to conventional shunting procedures. Closure of the ventricular septal defect was performed successfully in 2 patients at a later date.

1349 CONGENITAL ABSENCE OF DUCTUS ARTERIOSUS (ADA)

Newborn infants (NB) with cyanotic heart disease and reduced pulmonary flow are often ductus-dependent and improve with prostaglandin (PGE) infusion. However, PGE may fail if the ductus arteriosus is absent. The angiograms of 19 cyanotic NB (mean age 2.7 d) were analyzed. Group A consisted of 5 with ADA, and group B of 14 with patent ductus (PDA). All had pulmonary atresia complex with or without VSD. The diameters of aorta (ascending [AA], descending [DA], at diaphragm level [DDA]), right pulmonary artery (RPA), and left pulmonary artery (LPA) were obtained. Aortic size in A did not differ from those in B at all 3 levels. However, RPA and LPA were smaller in A than in B shown by mean pulmonary/aortic ratios: RPA/AA = 0.21 vs 0.389, RPA/DA = 0.38 vs 0.628, RPA/DDA = 0.41 v 0.64, LPA/AA = 0.20 vs 0.36, LPA/DA = 0.36 vs 0.60, LPA/DDA = 0.39 vs 0.605 (p value in all < 0.005), indicating impaired pulmonary artery growth in utero in pulmonary atresia or severe pulmonary stenosis with ADA. The angiograms in B showed PDA in all, and no increase of bronchial collaterals. In contrast, bronchial flow was abundant in A. Aortic valve stenosis was present in 3/5 in A but in 0/14 in B. ADA may be suspected clinically in cyanotic NB with reduced pulmonary flow if there is no response to PGE infusion and/or if aortic valve stenosis is also present. The diagnosis may be established angiographically based on non-visualization of the ductus, diminutive pulmonary arteries and abundant bronchial flow.

Staged surgical management of pulmonary atresia with diminutive pulmonary arteries

A particular problem in treatment is presented by patients who have atresia of the pulmonary valve and ventricular septal defect but whose pulmonary arterial tree is extremely diminutive. The pulmonary blood flow results from naturally occurring large bronchial collateral arteries. In the experience reported, satisfactory palliation was provided for all 3 patients in whom right ventricle--to--pulmonary artery continuity was constructed by placing a pericardial patch graft during open-heart operation. The ventricular septal defect was left unrepaired. Early evidence supports the hypothesis that, besides providing palliation of cyanosiis, the procedure may allow progressive enlargment of the hypoplastic pulmonary arteries. This offers hope that later second-stage completion of total repair, involving closure of the ventricular septal defect and ligation of any remaining large bronchial collateral arteries, may become feasible.

Tetralogy of Fallot with acquired pulmonary atresia and hypoplasia of pulmonary arteries: Report of surgical management in infancy

A perforated intracardiac prosthesis, and patch infundibuloplasty and annuloplasty were employed for the surgical management of an infant with tetralogy of Fallot, pulmonary atresia, and severe hypoplasia of the pulmonary arteries. This approach provides a means of decompression of the right ventricle in the immediate postoperative period. It allows sufficient systemic cardiac output while increasing pulmonary blood flow to relieve severe hypoxemia and cyanosis. In addition, the high risk of further compromise to the diminutive pulmonary arteries by a palliative shunt is avoided. A physiological basis for the use of this technique is discussed.