Biventricular Repair of Palliated Congenital Heart Disease: A Single Center Experience Background  There has been a dramatic reduction in mortality after complex congenital cardiac surgery [1].  The relationship between institutional volume and outcome is less clear and Gauvreau has suggested that institutionally-specific risk adjusted data should be developed [2].  Larger numbers of patients with Fontan circulation survive into adult life and some have a potentially viable biventricular circulation [3].  We present the re-evaluation of three cases where further surgical intervention was not originally recommended. Case 1  A 2-year-old female with pulmonary atresia, VSD and MAPCAs was deemed inoperable on two previous occasions with SaO 2 in the 50’s, Hb > 20 g/dl, cachexia, and on palliative care.  Investigation and Surgery: Based on repeat angiography findings (Fig. 1), unifocalization was performed with immediate improvement of SaO 2 to 90%.  She was discharged home on day 4 post surgery.  Follow up: The child has been asymptomatic since surgery (Fig. 2), has had two balloon dilatations due to stenosis of the peripheral PA and is awaiting complete repair. Case 2  A 3-year-male with unbalanced AVSD, hypoplastic RV (Fig. 3), situs solitus, persistent LSVC with absent right SVC, hemiaygos continuation of IVC to LSVC, and hepatic veins to the left of the mid point of the common atrium was considered unsuitable for complete repair.  He had a PA band placed at 3 months followed by Kawashima procedure at the age of 9 months.  He had limited activity, was pale, cyanotic with mild clubbing and SaO 2 70%.  Investigations: MRI, 3D echocardiography and cardiac catheterization  Severe bilateral PAVMs in the lungs with RV volume of 41 mL/m 2 on MRI  3D echocardiography confirmed mild hypoplasia and repairable AV valve (Fig. 4)  Surgery: Reconnection of RV to PA with 19 mm trifecta prosthesis, repair of AVSD and baffling the hepatic venous return to pulmonary circulation.  Follow up: At 1 year follow up, he had no cardiac symptoms or limitation of activity with SaO 2 of 100% on room air.  Perihepatic collaterals decompressing the IVC through the hepatic vein to RA were found, but pressures were unaffected by trial occlusion of IVC during follow up catheterization  Hemodynamics: RV 40/11, femoral artery 85/44, SVC mean 22, RA mean 13, MPA 28/18 mmHg Fig. 5: PA angiogram showing the APC Fontan Fig. 6: 3D TEE assessment of RV morphology Fig. 2: PA angiogram at 3 months following unifocalization procedure Fig. 1: Angiogram showing MAPCAs Fig. 4: 3D multiplanar review shows adequately sized ventricles with common AV valve Fig. 3: 2D TTE shows hypoplastic RV Marcus P. Haw MBBS, MD, FRCS, FECTS; Neal D. Hillman MD; Harikrishnan K. N. Kurup MBBS, MD; Allison J. Amidon MSN, RN, CPNP; Bennett P. Samuel MHA, BSN, RN; Joseph J. Vettukattil MBBS, MD, DNB, CCST, FRCPCH, FRSM, FRCP Grand Rapids, Michigan Case 3  A 30-year-old female with pulmonary atresia intact ventricular septum had RMBTS and division of PA at age 4 years and an APC Fontan at age 7 years (Fig. 5).  Indication for intervention:  Failing Fontan with massive RA, severe effort intolerance, atrial flutter, oxygen dependent and wheelchair bound, comorbid obesity (BMI 36.5) and Hepatitis C.  Investigations: MRI, cardiac catheterization, 3D TEE (Fig. 6)  Severe TR into Fontan pathway, RV volume index 118 mL/m 2  Hemodynamics: RV pressure 30/12, Fontan pathway pressure 14, LA pressure 11 mmHg, normal LV, RVEF 40%  Surgery: Takedown Fontan with TVR, RV to PA conduit and atrial reduction with Maze procedure.  Follow up: At 1.5 year follow up, she had optimal ventricular function, was living independently, able to swim ½ mile and workout in the gym 3x/week. BMI is still > 30. Conclusions  Improvements in imaging, intensive care and surgical techniques can offer more possibilities to complex patients with palliated or unoperated complex congenital heart disease.  Primary and revision surgery can be accomplished safely in selected cases [3].  Decisions for palliative care or palliative surgery should be reviewed critically, when new information becomes available, in the setting of a multidisciplinary forum.  Life long care of complex cases may involve a reversal of decision making from previous era’s.  This will involve an institutional review of risk and a period of time with the patient and family to explain the specific thinking behind any recommendation.  A successful outcome can be life changing [3]. References 1. Gaureau K. Reevaluation of the volume-outcome relationship for pediatric cardiac surgery. Circulation 2007;115:2599-601. 2. Pagel C, Utley M, Crowe S, et al. Real time monitoring of risk-adjusted paediatric cardiac surgery outcomes using variable life-adjusted display: Implementation in three UK centres. Heart. 2013;99:1445-50. 3. Sharma V, Burkhart HM, Cetta F, Hagler DJ, Phillips SD, Dearani JA. Fontan conversion to one and one half ventricle repair. Ann Thorac Surg 2012;94:1269-74. View publication stats View publication stats