Abstract Background Palliative atrial switch (PAS) procedures that reroute pulmonary and systemic venous drainage and leave a ventricular septal defect (VSD) open have been used in the treatment of deeply cyanotic patients who have severe pulmonary vascular obstructive disease (PVOD). Palliative atrial switch is beneficial for patients with transposition of the great arteries or other complex lesions with VSD who show higher arterial oxygen saturation in the pulmonary artery than in the aorta (transposition hemodynamics/unfavorable streaming). We reviewed the early and late results of PAS (Mustard, n=25; Senning, n=3) in patients at two institutions. Methods Between April 1965 and March 2000, PAS was performed in 28 cyanotic patients (18 male, 10 female). Median age was 10 years (range, 1 to 27). Mean preoperative pulmonary arterial pressure was 68 mm Hg (range, 30 to 121 mm Hg). Mean systemic arterial oxygen saturation was 65% (range, 47% to 80%). The majority of patients (95%) were in New York Heart Association (NYHA) functional class III or IV preoperatively. Results Overall early mortality was 21%; for patients after 1972 (n = 23), the early mortality was 8.7%. Mean follow-up was 8.3 years (maximum 20). Mean postoperative systemic arterial oxygen saturation was increased significantly to 88% ( p p = 0.002). Conclusions The PAS operation significantly improves systemic arterial oxygen saturation and quality of life in selected patients with transposition hemodynamics, VSD, and severe PVOD.
#1Amalia Elizari(NIH: National Institutes of Health)H-Index: 3
#2Jane Somerville(NIH: National Institutes of Health)H-Index: 26
: A 28-year-old female patient with complete transposition, ventricular septal defect and persistence of the arterial duct underwent a palliative arterial switch procedure in 1976 at 7 years of age. Therefore, she has survived for 22 years and lives a near normal life. She is married, has been counselled against pregnancy and has increasing cyanosis with the typical features of the Eisenmenger syndrome.
OBJECTIVE--To assess the clinical, functional state, and complications late (15-20 years) after palliative Mustard operation. DESIGN--Examination and evaluation of all patients presenting in adolescence and adult life after palliative Mustard operation for transposition of the great arteries and pulmonary vascular disease. SETTING--Grown-up Congenital Heart Unit specialised in the care of adolescents and adults with congenital heart disease, designated as having "quaternary" status within a tert...
Last. Edward L. Bove(UM: University of Michigan)H-Index: 1
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Abstract Failure to repair transposition of the great arteries and ventricular septal defect in the young infant results in the early development of pulmonary vascular occlusive disease. Complete repair, preferably by an arterial switch procedure and ventricular septal defect closure, may then not be possible. We report a palliative arterial switch procedure in a 5 1 / 2 -year-old patient with transposition, ventricular septal defect, and severe pulmonary vascular obstructive disease in whom pro...
A palliative operation to create intraatrial transposition of venous return without concomitant closure of an associated ventricular septal defect (palliative Mustard or Senning operation) has been used primarily for patients with a combination of transposition of the great arteries, ventricular septal defect, and pulmonary vascular obstructive disease. We evaluated the results in all 15 patients (median age 13 years) who had this procedure at the Mayo Clinic between 1973 and 1979. There were tw...
A device is disclosed that includes a memory bit cell, a first word line, a pair of metal islands and a pair of connection metal lines. The first word line is disposed in a first metal layer and is electrically coupled to the memory bit cell. The pair of metal islands are disposed in the first metal layer at opposite sides of the word line and are electrically coupled to a power supply. The pair of connection metal lines are disposed in a second metal layer and are configured to electrically cou...
A long-term follow-up study after 41 palliative Mustard operations for transposition of the great arteries and pulmonary vascular obstructive disease is presented. The operations were performed from 1973 to 1980. Mean pulmonary arteriolar resistance was 13.96 ± 6.7 Um 2 . A ventricular septa] defect was not closed in 34 patients; in 7 it was created at operation. There were three hospital and two late deaths. Survivors were followed up for 3 to 10 years (mean 76.7 ± 22.8 months). Before operatio...
Four cases of palliative Mustard or Senning repair for transposition of the great arteries (VGA) with ventricular septal defect (VSD), hypoplastic right ventricle, and superior-inferior ventricular configuration are presented. The palliative Mustard procedure—a Mustard repair without VSD closure—is usually reserved for patients with pulmonary vascular obstructive disease (PVOD). In such cases, VSD closure would result in left ventricular failure. Each of our four patients had normal or only slig...
Twenty-two patients with transposition of the great arteries with or without ventricular septal defect and one with double outlet right ventricle, d-malposition, and severe pulmonary vascular obstructive disease were treated surgically. All were cyanosed and had very limited exercise tolerance. Preoperatively, systemic arterial oxygen saturation (SaO2) varied from 45 to 79% (mean 65), haemoglobin was 13 to 23 g/dl (mean 19). Pulmonary arteriolar resistance was 6.4 to 35 units m2 (mean 17). In th...
Five patients with double outlet right ventricle, ventricular septal defect, pulmonary arterial hypertension and pulmonary vascular obstructive disease and three patients with complete d-transposition of the great arteries, ventricular septal defect, pulonary arterial hypertension and pulmonary vascular obstructive disease underwent an elective Mustard baffle operation. The ventricular septal defect was not closed. A large patent ductus arteriosus was divided in three patients. Seven of the eigh...
Abstract The tendency toward development of severe pulmonary vascular obstructive disease in patients with complete transposition of the great arteries and associated large ventricular septal defect has been well documented. The physiologic consequence of this process is a significant reduction in intercirculatory mixing, resulting in increasingly severe levels of systemic arterial hypoxemia, compensatory polycythemia and an associated significant increase in symptoms. Data indicate that definit...
Palliations for congenital heart anomalies are surgical procedures meant to offset major hemodynamic derangements, usually in preparation for staged repair. They treat symptoms but cannot stop the progression of the disease. Palliative operations for congenital heart anomalies are classified into procedures aimed at: (1) reducing pulmonary blood flow (pulmonary artery banding); (2) increasing pulmonary blood flow (several methods of systemic-pulmonary shunt); (3) enhancing mixing at atrial level...
ABSTRACTIntroduction: Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) in patients with congenital heart disease (CHD). It is characterised by a severe r...
BACKGROUND: Transposition of the great arteries is the most common cyanotic cardiac lesion in newborns. Transposition of the great arteries without surgical correction is fatal during the first year of life. Contemporary outcome studies have shown that survival rates after surgery are excellent and most patients live to adulthood. CASE SUMMARY: Woman with complex transposition of the great arteries with atrial and ventricular septal defects and subvalvular and valvular pulmonary stenosis, who ha...
Abstract Advances in the management of congenital heart disease (CHD) in children have resulted in growing numbers of adults with CHD. Pulmonary arterial hypertension related to CHD (PAH-CHD) is a common complication, affecting up to 10% of patients; and can arise even after successful and complete defect repair, with severe and potentially fatal consequences. Careful work-up in these patients is essential, particularly hemodynamic assessment, and can help define the most appropriate therapeutic...
Late referral of patients with complete transposition of the great arteries (TGA) is common in developing countries. However, outcomes of these patients who undergo surgical correction late remain unclear. To obtain favorable outcomes in these “late” patients, understanding of the surgical indications, management and specific surgical techniques is essential. Although the anatomical and hemodynamic features of ‘TGA- intact ventricular septum (IVS)’ and ‘TGA-ventricular septal defect (VSD)’ seen ...
Background:Transposition of the great arteries (TGA) is the most common cyanotic cardiopathy, with an incidence ranging between 0.2 and 0.4 per 1000 live births. Many patients not treated in the first few months of life may progress with severe pulmonary vascular disease. Treatment of these patients may include palliative surgery to redirect the flow at the atrial level.Objective:Report our institutional experience with the palliative Senning procedure in children diagnosed with TGA and double o...
Pulmonary hypertension (PH) refers to elevated pulmonary artery (PA) pressure (PAP), defined as mean PAP≥25 mm Hg. PH is not synonymous with pulmonary arterial hypertension (PAH), which specifies resistive PH characterized by elevated pulmonary vascular resistance (PVR) and normal pulmonary venous pressure. Approximately 5% to 10% of adults with congenital heart disease (CHD) have PH, and this subset of CHD patients is at higher risk of hospitalization and death.1–3 Understanding the epidemiolog...
La hipertension arterial pulmonar es una complicacion frecuente de las cardiopatias congenitas (CC). Es reconocido que las CC son las malformaciones mas frecuentes al nacimiento con una prevalencia que va de 6 a 8 por 1,000 recien nacidos vivos. En nuestro pais se calcula que cada ano nacen de 12,000 a 16,000 ninos con algun tipo de malformacion cardiaca. En los pacientes no corregidos con cortocircuitos de izquierda a derecha el aumento de la presion pulmonar provoca incremento en las resistenc...
The state of the pulmonary vascular bed impacts on the outcome of CHD. Both active vasoconstriction and pathological remodeling conspire to increase PVR. The combination of increased pulmonary blood flow and pulmonary artery pressure is a potent stimulator of pulmonary vascular disease. With the exception of a few high-risk malformations (e.g., d-TGA and VSD), permanent or progressive pathological changes seldom occur when malformations are corrected in the first 1‐2 years of life. Patients with...
Dramatic advances in the diagnosis and treatment of congenital heart disease (CHD), the most common inborn defect, has resulted in a growing population of adults with CHD. Eisenmenger syndrome (ES) represents the extreme form of pulmonary arterial hypertension associated with CHD, characterized by markedly increased pulmonary vascular resistance with consequently reversed or bidirectional shunting. While ES is a direct consequence of a heart defect, it is a fundamentally multisystem syndrome wit...