LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: OPTIONS FOR SURGICAL MANAGEMENT

Transcription

1 LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: OPTIONS FOR SURGICAL MANAGEMENT March 2017 Ritz Carlton, Riyadh, Saudi Arabia Zohair AlHalees, MD Consultant, Cardiac Surgery Heart Centre

2 LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: Obstruction to the left ventricular outflow tract (LVOTO) may be encountered at Valvar level 50% Subvalvar level 25% Supravalvar level 10% Or combined levels 15% It may be isolated or associated with other defects such as Coarctation of the aorta Aortic arch interruption Pulmonary stenosis Mitral Valve Abnormalities Ventricular Septal Defect VSD

3 LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: SHONE COMPLEX (Shone Syndrome, Shone Anomaly) Four obstructive anomalies that have the tendency to co-exist. Supramitral valve ring in the left atrium Parachute deformity of the MV Subaortic Stenosis Coarctation of Aorta Shone JD et al 1963

4 LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: Diffuse left ventricular outflow tract obstruction with 2 good ventricles + VSD

5 LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: LVOTO can be a spectrum which may be present at varying degrees at different levels with the HLHS being at the extreme end of the spectrum.

6 LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: Whether a VSD is present or not the severity of the obstruction in the left heart dictates the clinical presentation, surgical management and operative outcomes.

7 LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: Despite being one of the most common congenital cardiac abnormalities. The treatment of LVOTO particularly in the neonate and infant remains a significant challenge.

8 LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: Univentricular or Biventricular? [Wrong choice may mean early or late poor outcome or death!]

9 LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: Contraindication for Biventricular Repair: Small left ventricle < 20 mm/ BSA, inlet length < 25mm Narrow aortic valve ring < 5 mm Small MV orifice <9 mm Extensive fibroelastosis

10 LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: Those patients who have borderline LV need to be carefully assessed for suitability of single ventricle or biventricular repair based on: Morphometrics Functional parameters Hemodynamics Available surgical options Results of the personal / institutional experience

11 Conclusions. We have successfully achieved biventricular repair in most of the patients with hypoplastic left heart complex, a subset of patients with hypoplastic left heart syndrome. Some growth of the left ventricular structures was already observed at the time of hospital discharge. However, reoperation, particularly for left ventricular outflow tract obstruction, appears likely. Increasing experience will more accurately define predictive criteria for the feasibility of biventricular repair.

12 Conclusions: Moderate/large ventricular septal defect, unicommissural aortic valve, and hypoplastic mitral valve or left ventricle are independent risk factors for failure of biventricular repair for infants with multiple left heart obstructive lesions. Combinations of these risk factors may be useful in selecting surgical strategy.

13 LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: RELIEF OF LVOTO + VSD CLOSURE D. Kitchiner et al (Heart 72:3: Sep 1994) 202 Clinical Cases of LVOTO 65 patients (32%) had associated VSD 32 pts short segment fibromuscular obstruction 33 pts LVOTO Muscular Component + Fibrous Obstruction in 39% 17 pts posterior deviation -> obstruction above the VSD 16 pts overriding the aorta with concordant ventriculo-arterial connection -> obstruction below the VSD Incidence of aortic arch obstruction was higher in patients with deviated structure.

14 LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: TWO ADEQUATE VENTRICLES + VSD Surgical options: 1. Relief LVOTO + VSD Closure 2. Yasui Procedure (Norwood or DKS + Rastelli) 3. VSD Closure + Ross Procedure

15 LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: RELIEF OF LVOTO + VSD CLOSURE If morphology allows, this is considered best approach: Normal anatomy No conduits Can be performed even in the presence of fibroelastosis (septal myomectomy + resection of the fibrosis) Low reintervention rate

16 LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: RELIEF OF LVOTO + VSD CLOSURE Size of aortic annulus is the most determinant factor for this approach. Aortic Annulus Size < Patient s weight (kg) mm poor outcome Aortic Annulus Size > Patient s weight (kg) mm good outcome ] Yasutaka Hirata, Jan Quaegebeur et al. Annals Thoracic Surgery [Aug 2010] 90:2: ]

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21 LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: Early and midterm clinical outcomes of primary biventricular repair for patients with LVOTO and VSD demonstrate good results. The association with coarctation/aortic interruption increases risk. In selected patients maintaining a small ASD may improve hospital mortality. Freedom from reintervention for recurrent LVOTO is around 88%.

22 Mini Ross Konno VSD Patch Closure

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26 Alsoufi, AlHalees et al. European Journal of Cardio-thoracic Surgery 38 (2010)

27 LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: In summary, associated lesions rather than LVOT morphology have a more important impact on outcomes in infants with critical LVOTO. Both the Yasui and the Ross procedures can attain a biventricular repair in a subset of patients with severe LVOTO and with results comparable to those reported for aortic valvotomy in neonates and infants with critical AS.

28 RESULTS: Three of the 17 (18%) died within 30 days. There were no deaths in this series since Nine patients (38.9%) required one reoperation, 7 of which were for conduit stenosis, 1 for left ventricular outflow tract obstruction, and 1 for residual ventricular septal defect with left ventricle to right atrium shunt. Freedom from death at 10 years was 82% by Kaplan Meier estimate.

29 BACKGROUND:... 2 adequate-sized ventricles. This combines a Norwood arch reconstruction with a Rastelli operation establishing a biventricular repair. METHODS: Actuarial survival after initial operation was 100% at 1 year and 75% at 5 years. Actuarial freedom from reoperation or death after biventricular repair was 14% at 5 years. CONCLUSION: Reoperation after biventricular repair seems inevitable, mostly for conduit replacement.

30 RESULTS:... The actuarial survival at 10 years was 87.8%. Six patients underwent reoperation, including 5 conduit exchanges, 2 LVOT repairs and 2 aortic arch repairs. The freedom from reoperation for all causes at 5 and 10 years were 71.3 and 28.5%, respectively.

31 LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: SUMMARY For the question of VSD Closure + Relief of LVOTO vs the Yasui Procedure. It is not one or the other as long as we have 2 adequate size ventricles. The approach depends on the morphology of the lesions. Most important determining factor. size of the aortic annulus ability to adequately relief LVOTO commitment of the VSD pulmonary valve function With the Yasui approach reoperation is inevitable while with relief of LVOTO + VSD closure freedom from reintervention is around 80% at 10 years.

32 LEFT VENTRICULAR OUTFLOW OBSTRUCTION WITH A VSD: THANK YOU!