Assessment of ventricular size and function in congenitally corrected transposition of the great arteries

Graham, T.P.; Parrish, Mark D.; Boucek, Robert J.; Boerth, Robert C.; Breitweser, James A.; Thompson, Sandra L.; Robertson, Rose Marie; Morgan, James I.; Friesinger, Gottlieb C.

doi:10.1016/s0002-9149(83)80043-5

Cited by 111 publications

(31 citation statements)

References 19 publications

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“…Among these features is the propensity for atrioventricular conduction abnormalities 12 and for TV dysfunction [1][2][3] as well as the much-debated capability of the RV to function adequately in the systemic circulation over the course of a normal life span, regardless of the state of the TV. [13][14][15][16] Several investigators have reported that TV function in patients with CTGA appears to be adversely affected by surgical repair of associated lesions. 2,3,7,8 Anatomic distortion of a structurally abnormal TV by VSD closure has been implicated, 7 as has annular dilation after cardiopulmonary bypass-induced RV dysfunction or large preoperative leftto-right shunts.…”

Section: Discussionmentioning

confidence: 99%

Progressive Tricuspid Valve Disease in Patients With Congenitally Corrected Transposition of the Great Arteries

et al. 1998

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Background-The outcome of patients with corrected transposition of the great arteries (CTGA) is variably affected by associated intracardiac defects, tricuspid valve competence, and systemic right ventricular (RV) function. The relative importance of these factors in long-term outcome has not been evaluated. Methods and Results-Since 1958, 40 patients with CTGA were studied to determine risk factors for poor outcome,including age, open heart surgery, tricuspid insufficiency (TI), cardiac rhythm, pulmonary overcirculation, and RV dysfunction. Follow-up ranged from 7 to 36 years (mean 20 years). Intracardiac repair was performed in 21 patients; 19 were unoperated or had closed heart procedures. For the purposes of this study the designation TI s refers to at least moderately severe TI as delineated by echocardiogram and/or angiography. TI s was the only independently significant factor for death (Pϭ0.01), and in turn, only the presence of a morphologically abnormal TV predicted TI s (Pϭ0.03). Twenty-year survival without TI s was 93%, but only 49% with TI s . Poor long-term postoperative outcome was due to TI s in all but 1 patient; 20-year survival rates for operated patients with and without TI s were 34% and 90%, respectively (Pϭ0.002). Similarly, 20-year survival rates for unoperated patients with and without TI s were 60% and 100%, respectively, whether or not attempts to repair the TI were made (Pϭ0.08). Conclusions-TI s represents the major risk factor for CTGA patients; RV dysfunction appears to be almost always secondary to long-standing TI. Decisions related to surgical interventions with or without associated lesions must be strongly influenced by the status of the tricuspid valve. (Circulation. 1998;98:997-1005.)

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Section: Discussionmentioning

confidence: 99%

Progressive Tricuspid Valve Disease in Patients With Congenitally Corrected Transposition of the Great Arteries

et al. 1998

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“…54 A hemodynamic assessment of associated anomalies can be performed, including measurement of left and right heart pressures and pulmonary resistance. In older patients, coronary angiography is necessary before any operative intervention.…”

Section: Cardiac Catheterizationmentioning

confidence: 99%

Transposition of the Great Arteries

2006

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Abstract-Many patients with ventriculoarterial discordance have survived to adulthood. Those with complete transposition of the great arteries have often had an atrial switch procedure (Mustard or Senning operation) performed, which leaves the morphological right ventricle (RV) supporting the systemic circulation. RV failure and tricuspid regurgitation are common. Some patients may ultimately require cardiac transplantation. Sinus node dysfunction is increasingly common with longer follow-up, and some patients need pacemaker implantation. Atrial arrhythmias are frequent, and atrial flutter may be a marker for sudden death. Patients with an arterial switch procedure are also surviving to adulthood. Long-term problems include coronary stenoses, distortion of the pulmonary arteries, dilatation of the neoaortic root, and aortic regurgitation. Patients with congenitally corrected transposition have both atrioventricular and ventriculoarterial discordance and therefore also have a morphological RV and delicate tricuspid valve in the systemic circulation. Associated defects, such as abnormalities of the tricuspid valve, ventricular septal defect, and pulmonary stenosis, occur in the majority of patients. Heart block occurs with increasing age. Atrial arrhythmias occur frequently, and their occurrence should prompt a search for a hemodynamic problem. Progressive tricuspid regurgitation occurs with age and is associated with deterioration of RV function. Surgical treatment should be considered at the earliest sign of RV dilatation or dysfunction. All patients should be seen periodically in a center where expertise in the clinical evaluation, imaging, and hemodynamic assessment of adult congenital heart disease is available.

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“…In CCTGA, the dysfunction of the systemic ventricle occurs with increasing frequency in older patients; [2][3][4] Graham et al 4 reported long-term outcomes of 182 patients with CCTGA, which indicated that by age 45, 56% of the patients with associated lesions had moderate or severe systemic ventricular dysfunction, and 32% of the patients without associated lesions had this complication. They also reported that the strongest risk factors for clinical congestive heart failure and systemic ventricular dysfunction were systemic valve surgery, systemic AV valve regurgitation, significant arrhythmia, history of any open heart surgery, and pacemaker therapy.…”

Section: Discussionmentioning

confidence: 99%

“…4 It is considered that long-term exposure to systemic pressure is an etiology of systemic ventricular dysfunction in patients with CCTGA. 2 Hornung et al detected myocardial perfusion defects on radioisotope images in 5 patients with CCTGA who had not undergone surgery. 6 Fixed defects that suggested myocardial infarction were seen in all 5 and reversible myocardial ischemia was present in 4 of them.…”

Section: Discussionmentioning

confidence: 99%

“…1 The dysfunction of the systemic ventricle occurs with increasing frequency in older patients with CCTGA, independent of their commonly associated structural defects. [2][3][4] We report a patient with CCTGA undergone the partial ventriculectomy for his systemic ventricular dysfunction. (Circ J 2003; 67: 354 -356) …”

Section: Congenitally Corrected Transposition Of the Great Arteries Tmentioning

confidence: 99%

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Congenitally Corrected Transposition of the Great Arteries Treated by Partial Systemic Ventriculectomy.

Kobashi

Suwa

Nakamura

et al. 2003

Circ J

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42-year-old man was admitted to hospital with worsening dyspnea. At the age of 18, he underwent annuloplasty of the systemic AV valve for severe valvular regurgitation related to CCTGA at another hospital and did not have any problems on follow-up until age 33 when he visited hospital with paroxysmal nocturnal dyspnea. Because the systemic AV valve regurgitation had recurred to a severe degree and systemic ventricular contraction had decreased, he underwent systemic AV valve replacement and annuloplasty of the pulmonary AV valve. At age 39, atrial fibrillation with a rapid heart rate developed and could not be controlled with medications, so the His-bundle was cryoablated to block AV conduction and a permanent rate-adaptive pacemaker inserted intravenously. Although he was controlled in all pacemaker rhythm without tachycardia, his systemic ventricular function decreased and he was in New York Heart Association (NYHA) functional class 4 even after receiving digitalis, diuretics, angiotensin-converting enzyme inhibitor (ACEI) and other oral inotropic agents.His height was 185 cm and body weight was 75 kg. His blood pressure was 116/60 mmHg and the pulse rate was 60 beats/min and regular. Auscultation showed no significant murmur. Electrocardiograms revealed all pacemaker rhythm (VVI). Chest X-rays showed a cardiothoracic ratio of 58% and systemic atrial and ventricular enlargement. Echocardiography revealed enlargement of the systemic atrium and ventricle, and reduction of the systemic ventricular systolic function (the left atrial dimension was 4.7 cm, the end-diastolic dimension of the systemic ventricle was 8.6 cm and the fractional shortening was 14%) ( Table 1, Fig 1). Pulmonary wedge pressure and right-side pressures were elevated ( Table 2). The systemic ventriculogram showed diffused hypokinesis, but myocardial perfusion imaging using technetium-99m tetrofosmin indicated a severe perfusion defect in the lateral wall and the apex at rest.Because his symptoms could not be relieved by medical treatment, he was transferred to Shonan Kamakura General Hospital to undergo partial systemic ventriculectomy in February 1998. Under cardiopulmonary bypass (CPB) with heart beating and without aortic cross-clamping, the systemic ventricle was incised laterally from the apex to the base. There was neither mitral apparatus nor recognizable papillary muscle because of the previous valve replacement. Much of the anterolateral wall that was thin and akinetic on intraoperative palpation, and which corresponded to the defect on perfusion imaging, was excised in a tear-drop shape (7 cm wide and 12 cm long, 66 g in weight). Closure of the ventricle was done in 2 layers. The duration of CPB was 73 min and ventricular function recovered well without mechanical support. The resected myocardium had severe interstitial fibrosis (28.5%) and mild degeneration and hypertrophy of the myocardial cells.On echocardiography, the end-diastolic dimension of the systemic ventricle decreased to 5.7 cm, but the ejection fraction (EF) had not cha...

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Assessment of ventricular size and function in congenitally corrected transposition of the great arteries

Cited by 111 publications

References 19 publications

Progressive Tricuspid Valve Disease in Patients With Congenitally Corrected Transposition of the Great Arteries

Progressive Tricuspid Valve Disease in Patients With Congenitally Corrected Transposition of the Great Arteries

Transposition of the Great Arteries

Congenitally Corrected Transposition of the Great Arteries Treated by Partial Systemic Ventriculectomy.

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