Management of a Patient after the Bidirectional Glenn

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Management of a Patient after the Bidirectional Glenn Melissa B. Jones MSN, APRN, CPNP-AC CICU Nurse Practitioner Children s National Health System Washington, DC

No Disclosures

Objectives qbriefly describe the history and purpose of the BDG procedure qreview relevant preoperative data qdiscuss postoperative physiology and complications qreview challenges with mechanical support 3

History of the Cavopulmonary Anastamosis 1950 Carlon & colleagues demonstrated use of superior cavopulmonary shunt anastamosis in 8 dogs 1954 Shumacker described 2 patients who underwent a cavopulmonary anastamosis: TGA and truncus, both with high PVR, both died 1956 Meshalkin (Russia) reports 24 cases of superior cavopulmonary anastamosis 1 pulmonary atresia and 23 TOF 21/24 survived 1958 Glenn completed first successful cavopulmonary anastamosis in 1958 on 7year old in the US with TGAsurvived Konstantinov I, Alexi-Meskishvili, VV. Cavo-Pulmonary Shunt: From the First Experimentsto Clinical Practice. Ann Thorac Surg 1999;68:1100 6 4

Traditional Superior Cavopulmonary Anastamosis Anastomosis between SVC and RPA Reduces the volume load on the single ventricle Preserves myocardial and AV valve function Improves distribution of PBF and growth of pulmonary vascular bed Provides predictable Qp:Qs of 0.6-0.7:1 O 2 sats=75-85% 5

Comprehensive Stage II Hybrid Modification Review of Stage One Hybrid Modification Bilateral branch PA bands PDA stenting Atrial septostomy Comprehensive Stage Two Modification Removal of PDA stent Removal of PA bands Atrial septectomy Norwood/Arch reconstruction Cavopulmonary anastamosis http://www.nationwidechildrens.org/hypoplastic-left-heart-syndrome 6

Pre-Glenn Cardiac Catheterization Pulmonary artery pressure and anatomy Pulmonary vascular resistance Aortic arch AV-valve function Caval anatomy EDP/ventricular compliance Pulmonary vein O 2 sats 7

Single center retrospective analysis of 557 single ventricle patients who underwent Stage II palliation from 1998-2010 Ventricular dysfunction, AV-valve regurgitation and unbalanced AV septal defects were risk factors for survival to Fontan 8

Postoperative Cardiopulmonary Interactions Spontaneous breathing Negative intrathoracic pressure Positive pressure Positive intrathoracic pressure RA tm fl RA tm fl RAp RAp Increased venous return Decrease venous return 9

December 19, 2016

Postoperative Cardiopulmonary Interactions extubated December 19, 2016

Postoperative Problems: Cyanosis What makes up the aortic O2 sat? q Low MVO2 sat Anemia Increased consumption Low output qpulmonary vein desaturation CXR (ETT, edema, atelectasis, effusion, pneumo, etc) q Restrictive atrial septum q Decrease pulmonary blood flow Elevated PVR Anatomic obstruction in BDG circuit Poor ventricular function AV-valve regurgitation q V-V collaterals 12

Postoperative Problems: Cyanosis Spontaneous breathing will improve hemodynamics Ventilation is aimed at maintaining FRC and minimizing mean airway pressure to optimize oxygenation 13

Postoperative Problem: Cyanosis Relationship of PCO 2 and PO 2 in cyanotic BDG December 19, 2016

Postoperative Problem: Hypertension Unclear etiology Pain Catecholamine surge Intracranial hypertension Maybe be needed for cerebral perfusion? Hyperdynamic ventricle after removal of volume load Often patients are not naive to opiods December 19, 2016

BDG and Length of Stay 448 patients following S2P patients were discharged home between S1P and S2P median LOS 8days additional 3 days if performed between 2-4months of age Factors strongly influencing LOS (>14days)-multivariate analysis need for reoperation after S2P need for cardiac catheterization after S2P the use of non-oral forms of nutrition development of postoperative complications 16

Mechanical Support and BDG Evaluation of ELSO database to characterize difference between survivors and non-survivors 103 infants from 1999-2012, age 3mo-1 year Predictors of mortality inotropic requirement before ECMO duration of ECMO mechanical complications with the ECMO circuit renal failure pulmonary hemorrhage or pneumothorax 41% survived to hospital discharge 14% of those with neurologic injury

Ventricular Assist Device in Single-Ventricle Heart Disease and a Superior Cavopulmonary Anastomosis Retrospective review of patients supported with Berlin Heart after superior cavopulmonary shunts 4 patients reviewed Ventricular cannulation facilitated by excision of trabeculae and chords ¾ survived to transplant Niebler RA, Shah TK, Mitchell ME, Woods RK, Zangwill SD, Tweddell JS, Berger S, Ghanayem NS. Ventricular assist device in single ventricle heart disease and a superior cavopulmonary anastamosis. Artif Organs. 2015 Jul 6

December 19, 2016 Quick Case

Summary BDG circulation reduces volume load on the single ventricle, preserves myocardial and AV valve function and provide predictable Qp:Qs Spontaneous breathing is best for reducing the transpulmonary gradient and optimizing hemodynamics Cyanosis and hypertension are common postoperative problems The anatomy of the BDG present challenges for mechanical support, however ECMO and VAD are both options if medical management fails December 19, 2016