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Ventricular Septal Defect & Aortic Incompetence. Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery. VSD & Aortic Incompetence. 1. Definition VSD and AI syndrome includes hearts in which aortic incompetence is of congenital origin, although rarely
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Ventricular Septal Defect & Aortic Incompetence Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery
VSD & Aortic Incompetence • 1. Definition • VSD and AI syndrome includes hearts in which aortic • incompetence is of congenital origin, although rarely • present at birth, due to cusp prolapse or a bicuspid • aortic valve. The VSD is either doubly committed • subarterial or perimembraneous • 2. History • Laubry & Pezzi : Initial description in 1921 • Garamella, Starr : 1st operation in 1960 • Spencer, Trusler : Renewed publication of leaflet • reconstruction in 1973 preceded • by Frater
Morphology of VSD & AI • 1. Conoventricular (perimembraneous), juxtaaortic, juxtatricuspid • VSD are the most prevalent in Caucasian patients. • 2. Less commonly, the VSDs are in RV outlet and are juxtaaortic • and a few are juxtaarterial. • 3. Among Asians, VSDs in RV outlet and particularly juxtaarterial • VSDs are more prevalent. • 4. Most commonly the right cusp (60-70%) has prolapsed. • The noncoronary cusp prolapses in 10-15% both in 10-20%. • Uncommonly no cusp is prolapsing, but aortic valve is bicuspid • and incompetent. • 5. Variable RVOTO are present in many patients. • 6. Sinus of Valsalva adjacent to prolapsed leaflet is enlarged.
Clinical Features & Diagnosis • 1. The exact prevalence of leaflet prolapse with or • without AI in conoventricular VSDs is unknown(5%) • 2. Younger patients with mild AI • The signs of VSD dominate the clinical picture, but as • AI increases, the shunt decreases. • Such patients have a to-and-fro murmur. • 3. Assessed by noting possible presence of aortic leaflet • prolapse at cineangiography, echocardiography
Etiology of Valve Prolapse • Uncertain but ; • 1. Lack of support of the aortic sinus and • annulus by infundibular septum • 2. Structural defect in the base of the sinus • itself • 3. Hemodynamic influence during both systole • and diastole
Aortic Valve Prolapse • Pathogenesis • 1. Anatomic factors for normal competence • Commissary support from above • Leaflet support by diastolic apposition • Infundibular support from below (Van Praagh) • 2. Intrinsic structural abnormality • Progressive discontinuity between aortic valve • annulus and the aortic media (Yacoub) • 3. Hemodynamic factor • Venturi effect during early systole – turbulent • flow displace the cusp
Natural History of VSD and AI • 1. Unknown exact prevalence, but is related in part • to the age and rare before 2 years • 2. AI does not usually appear until 2-5 years of age. • 3. Once incompetence appears, it gradually increases • and within 10 years is usually severe. • 4. More severe because of additional volume load • from the VSD than the isolated lesion. • 5. Aneurysm of sinuses of Valsalva may develop as • part of the natural history in outlet type VSD.
Indications for Operation • 1. When a child with VSD first shows any signs of • development of prolapse, and the murmur of AI, • repair of VSD should be promptly accomplished. • 2. Even if prolapse has not occurred, juxtaarterial, • and RVoutflow juxaaortic VSD should be closed • to prevent prolapse. • 3. When AI is above moderate, the operation should • be undertaken promptly.
Technique of Operation • 1. When AI is trivial or absent, the VSD only is repaired • 2. When AI is significant, and often when mild, • the VSD and aortic valve is repaired. • 3. The aortic valve must usually be repaired or replaced • in adult when AI is moderate or severe. • * Trusler’s method of plication • * Frater stitch • * Triangular excision & reconstruction
Trusler Technique • Repair of prolapsed aortic valve cusp
Yacoub Technique • Repair of aortic valve in syndrome of VSD & aortic valve regurgitation
Results of Operation • 1. Survival • 2. Heart block • 3. Relief of aortic incompetence • 1) Preoperative severity • 2) Conoventricular (perimembraneous) VSD • 3) Bicuspid valve • 4) Old age • 4. Freedom from aortic valve replacement • 81% in 20 years