Pulmonary Branch Stenoses Research Articles

Palliative balloon angioplasty in a cat with right pulmonary arterial branch stenoses and concurrent absence of the left pulmonary artery

Unilateral absence of the left or right pulmonary artery with concurrent contralateral pulmonary arterial branch stenoses is an exceptionally rare disorder. We describe this anomaly in a cat with severe exercise intolerance and respiratory distress. Transthoracic echocardiography and angiography demonstrated the absence of the left pulmonary artery with concurrent right pulmonary branch stenosis. Palliative balloon angioplasty of the right pulmonary artery substantially reduced right ventricular systolic pressure load and alleviated the patient's clinical signs.

Endovascular stenting for treatment of pulmonary branch stenosis in adult

ObjectiveTo explore the curative effect and safety of endovascular stenting for treatment of pulmonary branch stenosis.MethodsTransthoracic echocardiography and right-sided haemodynamics revealed severe pulmonary hypertension in 2 cases (1 male and...

Encouraging results for the Contegra conduit in the problematic right ventricle–to–pulmonary artery connection

The Contegra conduit was developed for right ventricular outflow tract reconstruction. This report evaluates the Contegra conduit, with focus on certain subpopulations in which conduits are known to perform poorly (ie, patients with previous homograft conduits and infants). A retrospective review of 76 patients who had 77 Contegra conduits placed for right ventricular outflow tract reconstruction (January 2001 through August 2005) was completed. Characteristics include the following: median age of 1.6 years (range, 17 days-15.1 years), weight of 9.8 kg (range, 2.5-64.0 kg), and conduit diameter of 16 mm (range, 12-22 mm). Operations performed include right ventricular outflow tract reconstruction for pulmonary atresia-stenosis (n = 33), conduit exchange (n = 28), truncus repair (n = 7), primary conduit placement (n = 6), and the Ross procedure (n = 3). Seventy-nine percent were reoperations. There was no hospital mortality. Mean follow-up was 20 +/- 14 months. One-, 2-, and 3- year freedom from severe conduit regurgitation was 97%, 86%, and 81%, respectively, and freedom from severe conduit stenosis was 100%. Freedom from reoperation for conduit failure at 1 and 3 years is 98.3% and 93.1%, respectively. All conduit failures (n = 3) were for asymptomatic conduit pseudoaneurysms in the setting of multiple-level pulmonary branch stenoses. Survival at 3 years is 96%. Infants (n = 26) had a freedom from Contegra conduit failure at 3 years of 100%. Patients with previous homograft conduits (n = 26) had a freedom from Contegra conduit failure at 3 years of 100%. At midterm follow-up, the Contegra conduit remains a reliable, accessible, and easily implantable conduit for right ventricular outflow tract reconstruction. It appears to be the most promising conduit option for patients with previous homograft conduits and for infants.

Repaired Tetralogy of Fallot in the Adult

The majority of persons living with tetralogy of Fallot are now adults and may face a number of long-term cardiac problems that necessitate reoperation. These problems include pulmonary regurgitation, tricuspid regurgitation, ventricular tachycardia, atrial flutter and/or fibrillation, pulmonary artery branch stenoses, right ventricular aneurysms, right ventricular outflow tract obstruction, residual ventricular septal defects, and coronary artery disease. Management approaches to these potential problems are discussed. Issues related to genetics, pregnancy, infective endocarditis, insurability, and employment are also reviewed with specific reference to the individual with repaired tetralogy of Fallot.

Interventional catheterization in surgically treated patients with congenital heart disease.

Interventional catheterization is an alternative to surgery for some congenital heart defects. For other malformations, the surgeon and the interventionist will join in an effort to obtain an optimal result: the typical example is pulmonary atresia with VSD and aortopulmonary collaterals. In other cases, the cardiologist may be called upon to intervene with catheter techniques to correct sequelae or residual lesions after surgical correction, avoiding redo surgery. Most often, the task consists of opening stenoses by balloon dilatation and/or stenting the main targets being pulmonary artery branch stenoses, venous obstructions after Mustard procedure, and recoarctations. Whereas simple balloon dilatation of recoarctation often brings good results, stents are often needed to obtain optimal results in pulmonary branch stenoses. Stenting of pulmonary veins has been disappointing. Closing unwanted vessels and defects is another task for the interventional cardiologist after cardiac surgery. Here, the most frequent procedure is closing aortopulmonary collaterals in pulmonary atresia and VSD after corrective surgery. Advantages and limitations of these procedures are discussed.

Pulmonary artery abnormalities in tetralogy of Fallot and relation to late physical performance.

Preoperative angiograms were reviewed in 86 (52%) of 165 patients who underwent intracardiac repair of tetralogy of Fallot in 1966-1976. The median age at repair was 7 years (range 9 months-55 years). Hypoplasia, stenosis or atresia of the pulmonary and aortopulmonary collateral arteries were present in 58 patients (67%). Hypoplasia of the main pulmonary artery was associated with hypoplasia of the pulmonary artery branches. In patients who received a transannular patch the pulmonary arteries were smaller than in those without patch. Three of five pulmonary artery branch stenoses were located at the site of a previous palliative shunt. The postrepair right ventricular/left ventricular systolic pressure ratio (PRV/LV) measured in the operating room was compared with a predicted ratio based on pulmonary artery dimensions. High PRV/LV was associated with poor prognosis. In survivors the postrepair PRV/LV had fallen significantly 13-26 years later. Work capacity and pulmonary function at follow-up were not influenced by presence of pulmonary artery abnormalities. We conclude that such abnormalities, although common in patients with tetralogy of Fallot, did not per se influence physical performance in long-term survivors.

Intravascular stents for management of pulmonary artery and right ventricular outflow obstruction.

This study was performed to determine the efficacy of balloon-expandable stents in the treatment of branch pulmonary artery-stenoses and conduit stenosis in children. A total of eight stainless steel stents were implanted in seven patients. Three patients had tetralogy of Fallot with pulmonary artery stenosis following total correction, one patient had conduit stenosis following correction of transposition of the great arteries, one patient had intra-cardiac conduit stenosis after septation for single left ventricle, and two patients had pulmonary artery stenosis after Fontan operation. Six stents were placed in the branch pulmonary arteries, one in the extracardiac conduit, and one in the intracardiac conduit. The mean age at implantation was 13 +/- 3 years and the mean weight 37 +/- 12 kg. Follow-up time ranged from 0.3-2 years. The diameter of pulmonary arteries with stenoses increased from 5.6 +/- 2.2 mm to 10.6 +/- 1.8 mm (n = 7). The systolic pressure gradient decreased from 56 +/- 26 mmHg to 22 +/- 16 mmHg (n = 5). No embolization or thrombotic event has been noted. One stent placed in the intracardiac conduit was compressed and fractured. These data indicate that balloon-expandable stents are useful in the treatment of pulmonary artery branch stenoses and extracardiac conduit stenosis in children. The use of stents for intracardiac stenosis may result in stent fracture.

Intermediate-term clinical and hemodynamic results of the neonatal arterial switch operation for complete transposition of the great arteries

We prospectively evaluated 49 consecutive hospital survivors of the arterial switch operation for complete transposition and intact ventricular septum by clinical examination, echocardiography, cardiac catheterization, 12-lead and 24-h Holter ECG. The mean length of follow-up was 40 ± 18 months. Forty-six children are clinically asymptomatic without medication, 2 died due to coronary related left ventricular dysfunction 3 and 12 months after surgery, and 1 required reoperation because of severe bilateral pulmonary branch stenoses. Except for this case, cardiac catheterization ( n = 23) revealed a mean gradient of only 17 ± 8 mmHg between the right ventricle and distal pulmonary arteries. Left ventricular end-diastolic volume was within normal limits except for 2 cases with volumes slightly below normal, the mean ejection fraction was 78 ± 5%, and end-diastolic and end-systolic ventricular shapes were normal. The mean cardiac index was 4.14 ± 0.69 l/min/m 2. Left ventricular end-systolic wall stress to velocity of fiber shortening relation was normal in all cases examined ( n = 15), indicating normal myocardial contractility. Significant neoaortic valve insufficiency was never observed despite considerably enlarged aortic roots. Twenty-four-hour Holter ECG records ( n = 46) provided no evidence of serious atrial arrhythmias, especially sinus node dysfunction. These encouraging intermediate-term results make the arterial switch operation the treatment of choice at present, for neonates with simple transposition.

A useful projection for demonstrating the bifurcation of the pulmonary artery

The demonstration of the pulmonary bifurcation is important in order to exclude pulmonary branch stenoses. The origin of the right and left pulmonary arteries can be demonstrated in the anteroposterior plane if cranial angulation is used. Depending on the course of the left pulmonary artery, the origin of the left pulmonary artery may not be seen in spite of the maximal cranial angulation. On the lateral plane without tube angulation the origin left pulmonary artery is commonly superimposed on the origin of the right pulmonary artery. If maximum caudal angulation is added to the steep left anterior oblique view, not only the left, but also the origin of the right pulmonary artery can be seen.

Scimitar syndrome with peripheral left pulmonary artery branch stenoses

Partial anomalous pulmonary venous return most commonly involves the right pulmonary veins, with the site of connection most frequently the superior vena cava. Other sites of connection include the right atrium, inferior vena cava, superior vena cava plus right atrium, azygos vein, and coronary sinus.1-4 The pathologic features of a partial anomalous pulmonary venous drainage to the inferior vena cava associated with hypoplasia of the right lung were initially described by Parks in 1912. The term “scimitar syndrome” was coined by Neil1 et a1.2 in 1960 to describe the syndrome of partial anomalous pulmonary venous drainage of the right lung into the inferior vena cava, hypoplasia of the right lung with bronchial abnormalities and abnormal lobation, and partial systemic arterial supply to the right lung. Herein, we report a case of scimitar syndrome with an unusual associated vascular abnormality which presented a confusing clinical picture. A 19-year-old female with congenital heart disease was referred prior to a contemplated pregnancy for evaluation of a murmur. Her medical history was notable for pneumonia at the age of 4 years, at which time a heart murmur was first noted, and several recurrent bouts of pneumonia throughout her childhood and teenage years. She denied a history of cyanosis. Physical examination was notable for a prominent right ventricular heave and palpable third and fourth heart sounds. A loud grade IV/VI systolic murmur was heard, as well as a nearly pandiastolic murmur, both heard loudest over the entire left chest and only faintly audible over the right chest. The ECG revealed right axis deviation with right ventricular hypertrophy. The chest roentgenogram (Fig. 1) showed dextroposition of the heart and the typical scimitar configuration with a cardiothoracic ratio of 13.2122.6. The lung fields showed increased vascularity of the right lung. A lung scan showed increased perfusion of the right lung compared with that of the left (Fig. 2). Cardiac catheterization was carried out and revealed moderate elevation of right heart pressures. The main pulmonary artery pressure was 55/20 with a mean of 35 mm Hg. Pulmonary capillary wedge pressure was normal. Oximetry and green dye curves were compatible with partial anomalous right pulmonary venous return with an oxygen step-up in the inferior vena cava, 2 cm below its junction with the right atrium. An anomalous pulmonary vein emptying into the