Long-term follow-up of truncus arteriosus repaired in infancy: A twenty-year experience

Rajasinghe, Hiranya A.; McElhinney, Doff B.; Reddy, V. Mohan; Mora, Bassem N.; Hanley, Frank L.

doi:10.1016/s0022-5223(97)70259-9

Cited by 155 publications

(101 citation statements)

References 13 publications

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“…Patients with TA and del22q11.2 have a higher incidence (10-15%) of discontinuity of the pulmonary arteries Marino et al, 2002] (TA Type A3, according with Van Praagh classification), the ''duct dependent'' pulmonary artery being usually the left one in association with left aortic arch [Calder et al, 1976]. Other specific features associated with del22q11.2 include dysplastic truncal valve (30%) leading to incompetence and/or stenosis [Marino et al, 1998;Rajasinghe et al, 1997], interrupted aortic arch (10-20%) [Sano et al, 1990], and coronary artery abnormalities (18%) [de la Cruz et al, 1990].…”

Section: Truncus Arteriosusmentioning

confidence: 99%

Cardiac defects and results of cardiac surgery in 22q11.2 deletion syndrome

Carotti

Digilio

Piacentini

et al. 2008

Dev Disabil Res Revs

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Specific types and subtypes of cardiac defects have been described in children with 22q11.2 deletion syndrome as well as in other genetic syndromes. The conotruncal heart defects occurring in patients with 22q11.2 deletion syndrome include tetralogy of Fallot, pulmonary atresia with ventricular septal defect, truncus arteriosus, interrupted aortic arch, isolated anomalies of the aortic arch, and ventricular septal defect. These conotruncal heart defects are frequently associated in this syndrome with additional cardiovascular anomalies of the aortic arch, pulmonary arteries, infundibular septum, and semilunar valves complicating cardiac anatomy and surgical treatment. In this review we describe the surgical anatomy, the operative treatment, and the prognostic results of the cardiac defects associated with 22q11.2 deletion syndrome. According to the current literature, in patients with tetralogy of Fallot with/without pulmonary atresia and truncus arteriosus, in spite of the complex cardiac anatomy, the presence of 22q11.2 deletion syndrome does not worsen the surgical prognosis. On the contrary in children with pulmonary atresia with ventricular septal defect and probably in those with interrupted aortic arch the association with 22q11.2 deletion syndrome is probably a risk factor for the operative treatment. The complex cardiovascular anatomy in association with depressed immunological status, pulmonary vascular reactivity, neonatal hypocalcemia, bronchomalacia and broncospasm, laryngeal web, and tendency to airway bleeding must be considered at the time of diagnosis and surgical procedure. Specific diagnostic, surgical, and perioperative protocols should be applied in order to provide appropriate treatment and to reduce surgical mortality and morbidity.

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Section: Truncus Arteriosusmentioning

confidence: 99%

Cardiac defects and results of cardiac surgery in 22q11.2 deletion syndrome

Carotti

Digilio

Piacentini

et al. 2008

Dev Disabil Res Revs

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“…Among hospital survivors, Rajasinghe and colleagues 130 reported an actuarial survival rate of 83% at 15-year follow-up. Virtually all the patients had reoperation by 10 to 12 years, however, with a median time to reoperation of 5.1 years.…”

Section: Following Up the Postoperative Patientmentioning

confidence: 99%

“…134 Otherwise, most reintervention is surgical. 135 Adult patients with good surgical results and no complications often have minimal symptoms, 130 and some can be active. Successful pregnancy has been reported, although it is discouraged given the high incidence of chromosomal 22q11 deletions in this patient population.…”

Section: Bashorementioning

confidence: 99%

Adult Congenital Heart Disease

Bashore

2007

Circulation

128

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It has variably been estimated that between 800 000 and 1 million patients with congenital heart disease are now adults and that by 2010 more patients older than 16 years will undergo surgery for congenital heart disease than those younger than 16 years. 1 This review will focus on the clinical aspects of identifying and caring for the portion of the unique population that have right ventricular (RV) outflow tract lesions. Clinical Morphology of the RV Outflow TractThe left ventricle (LV) and RV both have inlet, apical, and outlet portions, although they differ considerably. 2 The inlet of the RV includes the tricuspid valve, and it is separated from the pulmonary valve (PV) by the crista supraventricularis. This structure results in the PV being raised upward. The apical portion of the RV has coarse trabeculations. As shown in Figure 1, the septum is home to a large septomarginal (septal) trabeculation that divides into the anterior and posterior limbs in the outflow tract. A series of septoparietal trabeculations arise along the free wall. The crista supraventricularis inserts between these limbs and is made up of 3 components: the muscular outlet septum, the ventriculoinfundibular fold, and the inner curvature of the RV. 3 The small part that separates the RV and LV cavities is the outlet or conal septum. A loop including the septal trabeculation, the moderator band, septoparietal trabeculations, and the outlet septum can be conceptualized as circling the RV outflow tract. The PV is supported by the circular muscular infundibulum rising outside the RV proper. Abnormalities involving the RV outflow tract involve malpositioning of 1 or more of these structures.

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“…Conduit replacement is eventually necessary in all cases because of homograft valvular stenosis due to shrinkage, distal anastomosis stenosis, conduit calcification, proximal hood aneurysm and somatic outgrowth [11]. Rajasinghe et al [12] and Tlaskal et al [13] in their studies of long-term follow-up of PTA after surgical correction reported a similar high incidence of conduit reoperations. In PTA type A1 and A2, Barbero-Marcial et al [14] and Nemoto et al [15] have described alternative techniques of right ventricle to pulmonary artery continuity established by direct anastomosis.…”

Section: Discussionmentioning

confidence: 99%

Long-Term Follow-Up of Persistent Truncus Arteriosus: Kuwait Experience

Selvan

Uthaman²,

Abushaban

et al. 2011

Med Princ Pract

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Objective: To evaluate the long-term results of patients in Kuwait who were operated for persistent truncus arteriosus (PTA). Subjects and Methods: The following data were collected for retrospective analysis from 24 medical records of consecutive patients with PTA in Kuwait between August 1993 and August 2009: demographics, morphology, management and outcome. Major associated abnormalities included interrupted aortic arch in 1 patient and abnormal coronary artery anatomy in 2. Results: Of the 24 patients, 16 underwent total intracardiac repair. The age at operation ranged from 15 days to 5 years (mean 166.19 ± 438.63 days) and weight ranged from 2.5 to 15 kg (mean 4.3 ± 3.01 kg). The right ventricle to pulmonary artery continuity was established with aortic homograft in 11, pulmonary homograft in 4 and by implantation of a Contegra conduit in 1 patient. Four patients had moderate truncal valve regurgitation requiring concomitant truncal valve repair. After a mean follow-up period of 81.81 ± 61.58 months (range 3–166) there was no death. Eight of the 16 (50%) patients underwent redo homograft operations. One patient who had concomitant truncal valve repair subsequently underwent aortic valve replacement. Conclusion: The data showed that complete repair of PTA in the neonatal and early infancy period was the treatment with the best potential for survival. The homograft remained one of the conduits of choice to establish continuity between the right ventricle and the pulmonary artery in spite of the high incidence of conduit redo operations.

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Long-term follow-up of truncus arteriosus repaired in infancy: A twenty-year experience

Abstract: Ten- to 20-year survival and functional status are excellent among infants undergoing complete repair of truncus arteriosus. Conduit replacement or revision is almost inevitably necessary in this group of patients.

Cited by 155 publications

References 13 publications

Cardiac defects and results of cardiac surgery in 22q11.2 deletion syndrome

Cardiac defects and results of cardiac surgery in 22q11.2 deletion syndrome

Adult Congenital Heart Disease

Long-Term Follow-Up of Persistent Truncus Arteriosus: Kuwait Experience

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