Pulmonary Valve Regurgitation and Branch Pulmonary Artery Stenosis

BACKGROUND AND OBJECTIVES: Repair of tetralogy of Fallot (TOF) with monocusp pulmonary valve reconstruction prevents pulmonary regurgitation (PR) for a variable period. Since postoperative outcome is governed by PR and right ventricular function, we sought to assess the severity of pulmonary regurgitation and right ventricular outflow (RVOT) gradient in the immediate postoperative period and at 1 year and attempted to identify the anatomical substrates responsible for adverse outcomes...

BACKGROUND: We sought to improve understanding of the diastolic prolongation of forward flow seen through a unilateral branch pulmonary artery (PA) stenosis. METHODS AND RESULTS: Of patients studied by cardiovascular magnetic resonance (CMR) for congenital heart disease, we reviewed right and left PA flow to find 10 cases with a diastolic prolongation of flow in a stenosed branch PA. They were aged 20 years (median, range = 14-40 years, 7 males). Seven had transposition of the great arteries corrected by arterial switch (TGA-AS) and 3 had repaired tetralogy of Fallot (rToF)...

Biventricular repair in right atrial isomerism is rarely feasible due to associated anomalies of venous connection, ventricular imbalance, nonroutabilty of the interventricular communication, a common atrioventricular junction, and inadequate pulmonary arterial branches. These patients are also often not ideal for univentricular repair due to some of the above associations. We describe a novel surgical technique that was utilized in such a patient for biventricular repair of a child with right atrial isomerism with total anomalous pulmonary venous connection, regurgitant common atrioventricular valve, hypoplastic left ventricle, nonroutable ventricular septal defect, and pulmonary stenosis...

In tetralogy of Fallot with absent pulmonary valve, pulmonary stenosis and regurgitation results in significant pulmonary artery dilatation. Branch pulmonary artery dilatation often compresses the tracheobronchial tree, causing fluid trapping in fetal life and air trapping and/or atelectasis after birth. Prenatal diagnosis predicts poor prognosis, which depends on the degree of respiratory insufficiency from airway compromise and lung parenchymal disease after birth. Fetal magnetic resonance imaging (MRI) has been useful in evaluating the effects of congenital lung lesions on lung development and indicating severity of pulmonary hypoplasia...

Right ventricular (RV) volume overload secondary to pulmonary regurgitation is common in patients after initial repair of tetralogy of Fallot (TOF) and is associated with adverse long-term outcomes. The objective of the present study was to determine the effect of branch pulmonary artery stenosis on the RV volume in patients with repaired TOF. We reviewed 178 cardiac magnetic resonance imaging studies in patients with repaired TOF. We defined bilateral stenosis as a Nakata index of ≤200 mm(2)/m(2) and concordant branch pulmonary artery cross-sectional area, unilateral stenosis as 1 branch pulmonary artery cross-sectional area ≤100 mm(2)/m(2) and 1 branch pulmonary artery cross-sectional area >100 mm(2)/m(2), and restrictive physiology as prograde main pulmonary artery diastolic flow...

OBJECTIVE: Chronic pulmonary regurgitation (PR) has deleterious effects on right ventricular (RV) function in repaired tetralogy of Fallot (ToF). However, there are little data regarding right ventricular outflow tract (RVOT) contractile dysfunction in response to chronic PR and on both RV and LV volumes and function. METHODS: We retrospectively identified consecutive patients with PR who were referred for magnetic resonance imaging quantification of "free PR" detected on echocardiography between 2003 and 2008...

After having undergone surgical correction at an early age, many patients with tetralogy of Fallot develop long-term complications including progressive pulmonary regurgitation and peripheral pulmonary stenosis. A high percentage of these patients need to undergo a second operation in their adolescence or early adulthood. If simultaneous treatment of both pulmonary regurgitation and peripheral pulmonary stenosis is warranted, a complete surgical approach has several disadvantages. We describe four cases of Fallot patients with severe pulmonary regurgitation and peripheral pulmonary stenosis who were treated using a hybrid approach involving surgical implantation of a pulmonary homograft and peroperative stenting of the pulmonary artery...

OBJECTIVES: We sought to investigate whether differential branch pulmonary artery (BPA) regurgitation correlates with differences in BPA anatomy and physiology. BACKGROUND: Patients with repaired conotruncal anomalies such as Tetralogy of Fallot frequently have residual BPA stenosis or BPA size differences. Previous reports have demonstrated an increased left pulmonary artery (LPA) regurgitant fraction (RF) in these patients. METHODS: We retrospectively reviewed 76 consecutive cardiac magnetic resonance (CMR) studies for BPA size and phase-contrast magnetic resonance data, including 13 consecutive patients who underwent both CMR and catheterization...

With prevalences ranging from 0.26 to 0.8‰ of all live births tetralogy of Fallot (TOF) is the most common congenital heart disease with primary cyanosis. Due to improvements in surgical techniques, nearly all patients can nowadays expect to reach adulthood. After surgical repair, pulmonary regurgitation (PR) occurs in almost every child and is an important contributing factor in long-term morbidity and mortality. Cardiac magnetic resonance imaging is well established for functional assessment and flow measurements and is an ideal tool for serial post-surgical follow-up examinations, as it is non-invasive and does not expose patients to ionizing radiation...

BACKGROUND: Transannular patch (TAP) repair of tetralogy of Fallot often results in significant right ventricular outflow tract (RVOT) dilation and distortion. We hypothesized that insertion of Melody valves into the proximal right and left branch pulmonary arteries (PAs) would reduce pulmonary regurgitation fraction (PRF) in an ovine model of pulmonary insufficiency and dilated RVOT. METHODS AND RESULTS: Ten sheep underwent baseline cardiac catheterization, surgical pulmonary valvectomy, and TAP placement...

BACKGROUND: The grading of pulmonary regurgitation (PR) severity by two-dimensional (2D) and Doppler echocardiography is not standardized. Cardiovascular magnetic resonance imaging is the clinical gold standard for PR quantification. The purpose of this study was to determine the best 2D and Doppler echocardiographic predictors of severe PR. METHODS: Thirty-six patients with tetralogy of Fallot or pulmonary valve stenosis with prior pulmonary valvuloplasty or transannular or subannular patch repair underwent 2D and Doppler echocardiography and cardiovascular magnetic resonance...

AIMS: To determine pregnancy outcome and risk factors for adverse events in women with congenital heart disease (CHD) and residual haemodynamic right ventricular (RV) outflow tract (RVOT) lesions. METHODS AND RESULTS: Pregnancy outcome data for women with CHD and residual RVOT lesions have been recorded since 2001. There were 76 pregnancies in 47 women that continued beyond 24 weeks gestation. At conception 20% had RVOT obstruction, 32% had pulmonary regurgitation (PR) and 49% had mixed RVOT obstruction and PR...

OBJECTIVE: The objective of this study was to evaluate the long-term outcome of total repair for tetralogy of Fallot. METHODS: Between April 1986 and December 2007, a total of 734 patients underwent total repair for tetralogy of Fallot. There were 444 males and 290 females. The median age and weight were 17.2 months (0.4-329.6 months) and 9.5kg (2.6-53.5kg). The median follow-up duration was 150.2 months (1.9-356.2 months). RESULTS: There were 27 early deaths (3...

OBJECTIVES: This study was designed to report a novel indication for percutaneous pulmonary valve implantation in patients with previous right ventricular outflow tract (RVOT) patch. BACKGROUND: Current indications for percutaneous pulmonary valve implantation are limited to patients who had pulmonary valve stenosis and/or regurgitation in a right ventricle-to-pulmonary artery conduit. Percutaneous pulmonary valve implantation has not been previously reported in patients with severe pulmonary valve regurgitation following repair of tetralogy of Fallot (TOF) using RVOT patch...

BACKGROUND: We analyzed the results in two centers of using bovine jugular vein graft for right ventricular outflow tract reconstruction. METHODS: From April 1999 to July 2005, 133 children with a median age of 30.9 months (range, 4 days to 19 years) underwent graft implantation. Echocardiography was performed during follow-up and retrospectively reviewed. RESULTS: Nongraft-related early mortality occurred in 8 patients. Late mortality occurred in 11 patients, 2 late deaths were graft related (endocarditis)...

Takayasu arteritis (TA) is a rare chronic vasculitis primarily involving the aorta and its main branches. The disease affects women much more frequently than men, the ratio being 8:1. The onset occurs in the teenage years, always before age of 40. Aortic regurgitation is rare. The pulmonary artery stenosis may also rarely occur. We report the case of a 49-year old female patient with Takayasu arteritis who presented with severe left pulmonary trunk stenosis resulting in pulmonary hypertension, severe aortic regurgitation due to the dilatation of the ascending aorta, mitral insufficiency, critical left renal artery stenosis, and probably with left main coronary artery stenosis...

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...

This case report describes the anesthetic management of a 32-year-old parturient with combined severe mitral regurgitation and moderate mitral stenosis, complicated by fast atrial fibrillation. The advantageous effects of epidural analgesia during labor and vaginal delivery and the importance of invasive monitoring are discussed. We also report the rare complication of right bundle branch block related to the use of a pulmonary artery catheter.

Repaired tetralogy of Fallot (rTOF) has an excellent long-term prognosis; however, survival is somewhat less than normal. Of all the residual lesions and sequellae after rTOF, pulmonary regurgitation (PR) is the most important, correlating with right ventricular (RV) size, exercise intolerance and serious ventricular arrhythmias. Pulmonary valve replacement (PVR) has beneficial effects on RV size and function, provided it is performed early, before irreversible RV dysfunction ensues. Moreover, PVR is associated with an improvement in patients' symptoms and exercise tolerance and combined with arrhythmia surgery (cryoablation) it leads to a dramatic decrease in the incidence of fatal ventricular arrhythmias...

OBJECTIVE: To describe aspects of the natural history and pathophysiology of giant low pressure pulmonary artery aneurysms and to propose potential surgical strategies. DESIGN: Cross sectional retrospective review. SETTING: Supraregional tertiary referral centre. PATIENTS: All adult patients referred for assessment of giant pulmonary artery aneurysm retrospectively identified from the Mayo Adult Congenital Heart Disease Clinic database...