Anatomic correction of the syndrome of prolapsing right coronary aortic cusp, dilatation of the sinus of valsalva, and ventricular septal defect

Yacoub, Magdi H.; Khan, Hasnat; Stavri, G; Shinebourne, E A; Radley-Smith, R

doi:10.1016/s0022-5223(97)70321-0

Cited by 103 publications

(59 citation statements)

References 14 publications

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“…Such patients raise the concern that progressive deformity of the right coronary cusp will lead to significant AR. 13,14 In the patients of the present series, the deformity did not progress, and in only 1 of the 5 was it complicated by AR that did not progress for 9 years after the onset. We detected trivial AR in another patient who had a tiny jet across the outlet septum; however, there was no associated abnormality of the right coronary cusp.…”

Section: Discussionmentioning

confidence: 52%

Incidence of Spontaneous Closure of Outlet Ventricular Septal Defect.

et al. 2001

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Section: Discussionmentioning

confidence: 52%

Incidence of Spontaneous Closure of Outlet Ventricular Septal Defect.

et al. 2001

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“…However, once the aortic lesion becomes evident, sophisticated valve reconstruction techniques need to be taken into consideration [Bonhoeffer et al, 1992;Yacoub et al, 1997].…”

Section: Ventricular Septal Defectmentioning

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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“…Yacoub et al [17] reported that a lack of continuity between the aortic media, annulus of aortic valve and the ventricular septum is the basic structural abnormality in patients with any type of VSD complicated by aortic valve prolapse and regurgitation. However, it does not entirely explain why most large perimembranous defects, that are closely related to the aortic valve and deficient in its septal support, are less frequently associated with aortic valve prolapse with or without regurgitation, than are subarterial VSDs with similar anatomy.…”

Section: Discussionmentioning

confidence: 99%