SHA24/043005

FAILING SINGLE VENTRICLE MICHEL N. ILBAWI, M.D. The Heart Institute for Children Chicago, Illinois

FAILING SINGLE VENTRICLE Fontan operation is a palliative procedure based on grossly abnormal hemodynamic principles.

Therefore In order to maximize its benefits, every attempt should be made preoperatively, intraoperatively and postoperatively to maintain hemodynamics as close to normal as possible.

THE HEART INSTITUTE FOR CHILDREN

THE HEART INSTITUTE FOR CHILDREN

FAILING SINGLE VENTRICLE WHAT CAN FAIL AND WHAT CAN WE DO ABOUT IT?

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FAILING SINGLE VENTRICLE • • • • • •

Arrhythmias Cyanosis/Desaturation Myocardial dysfunction Protein losing enteropathy Plastic bronchitis Renal/hepatic failure

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FAILING SINGLE VENTRICLE WHAT CAN BE DONE INTRAOPERATIVELY? • Selection of the Procedure – Atriopulmonary connection – Lateral tunnel connection – Extracardiac connection

• Fenestration THE HEART INSTITUTE FOR CHILDREN

THE HEART INSTITUTE FOR CHILDREN

THE HEART INSTITUTE FOR CHILDREN

FAILING SINGLE VENTRICLE WHAT CAN BE DONE INTRAOPERATIVELY? • Myocardial Protection – Beating heart – Continuous cardioplegia

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FAILING SINGLE VENTRICLE WHAT CAN BE DONE INTRAOPERATIVELY? • Aggressive management of atrioventricular valve pathology.

• Aggressive relief of any systemic outflow obstruction.

– Coarctation – Aortic/subaortic stenosis THE HEART INSTITUTE FOR CHILDREN

FAILING SINGLE VENTRICLE ARRHYTHMIAS • Interatrial reentrant tachycardia • Atrial fibrillation • Sinus node dysfunction

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FAILING SINGLE VENTRICLE ARRHYTHMIAS EARLY AND AGGRESSIVE MANAGEMENT • Conversion to extracardiac Fontan • MAZE operation • Placement of antitachycardia pacemakers

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THE HEART INSTITUTE FOR CHILDREN

THE HEART INSTITUTE FOR CHILDREN

FAILING SINGLE VENTRICLE RESULTS/ARRHYTHMIA SURGERY N = 62 PTS.

HOSPITAL DEATH

0/62 (0%)

ARRHYTHMIA CONTROLLED

58/62 (6.5%)

LATE DEATH

1/62 (1.6%)

TRANSPLANT

2/62 (3.2%)

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FAILING SINGLE VENTRICLE PULMONARY AND SYSTEMIC THROMBOEMBOLISM

MECHANISMS • • • • • •

Sluggish nonpulsatile flow/low c.o.p.

Clotting factors abnormalities Presence of prosthetic material Arrhythmias/lack of AV synchrony Ligated pulmonary artery stump Left atrial/ventricular focus BOTH PRO AND ANTI THROMBOTIC FACTORS

FAILING SINGLE VENTRICLE THROMBUS FORMATION Fontan Circuits Pulmonary Atrium Pulmonary Artery

48% 48% 2%

FAILING SINGLE VENTRICLE PULMONARY AND SYSTEMIC THROMBOEMBOLISM MANAGEMENT • Acute thrombosis • Chronic thromboembolism TO ANTIGOAGULATE OR NOT

FAILING SINGLE VENTRICLE ANTICOAGULATION ACUTE: – Low dose heparin

CHRONIC: – Residual shunts – Arrhythmias

TO ANTIGOAGULATE OR NOT CONTROVERSY GOES ON

FAILING SINGLE VENTRICLE ANTICOAGULATION RESULTS 217 PTS. ACCUTE THROMBOSIS

1/217

CHRONIC THROMBOSIS

0/217

THROMBOEMBOLISM

1/217

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FAILING SINGLE VENTRICLE CYANOSIS MECHANISMS •Systemic to pulmonary venous collaterals •Pulmonary arteriovenous malformation •Residual interatrial communication •Lateral tunnel shunt •Thebesian veins shunt •Pulmonary pathology THE HEART INSTITUTE FOR CHILDREN

FAILING SINGLE VENTRICLE CYANOSIS MANAGEMENT •Embolization of collaterals •Revision of Glenn or Fontan •Improving transpulmonary gradient •Closure of interatrial communication •Closure of fenestration THE HEART INSTITUTE FOR CHILDREN

FAILING SINGLE VENTRICLE MYOCARDIAL DYSFUNCTION MECHANISMS •Preoperative cyanosis/volume or pressure overload.

•Single ventricle with inadequate adaptation to systemic circulation.

RV vs. LV. •Lack of adaptation to Fontan circulation.

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FAILING SINGLE VENTRICLE MYOCARDIAL DYSFUNCTION MECHANISMS •Atrioventricular valve dysfunction.

•Inadequate myocardial protection.

•Residual systemic outflow obstruction.

•Arrhythmias.

THE HEART INSTITUTE FOR CHILDREN

FAILING SINGLE VENTRICLE MYOCARDIAL DYSFUNCTION MANAGEMENT •Surgical treatment of residual lesions •Medical management of failing myocardium Milrinone Afterload reduction B-Blockers Resynchronization therapy Mechanical support THE HEART INSTITUTE FOR CHILDREN

FAILING SINGLE VENTRICLE RESYNCHRONIZATION THERAPY • Prolonged QRS • Segmental dysynchrony

WHERE TO PLACE THE PACEMAKER ELECTRODS THE HEART INSTITUTE FOR CHILDREN

FAILING SINGLE VENTRICLE PROTEIN-LOSING ENTEROPATHY Mechanism Unknown •Venous stasis •Venous hypertension •Lymphatic problem Low Cardiac Output Inflammatory Process THE HEART INSTITUTE FOR CHILDREN

FAILING SINGLE VENTRICLE PROTEIN-LOSING ENTEROPATHY • • • •

Hypoalbuminemia Abnormal Ca++ metabolism Abnormal coagulation Lymphopenia/immunodeficiency

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FAILING SINGLE VENTRICLE PROTEIN-LOSING ENTEROPATHY General Management • • • • •

Fluid management Calcium/Diet Fenestration Optimize C.O.P. Transplantation

FAILING SINGLE VENTRICLE PROTEIN-LOSING ENTEROPATHY Medical Management • • • •

Budesonide Sildenafil Systemic steroids Subcutaneous heparin

FAILING SINGLE VENTRICLE HEPATIC DYSFUNCTION MECHANISM

• PreFontan hypoxemia • Perioperative injury • Chronically elevated venous pressure • Desaturation • Reduced hepatic blood flow

OUTCOME

• Cardiac Cirrhosis • Hepatic neoplasms • Coagulopathy

FAILING SINGLE VENTRICLE PLASTIC BRONCHITIS Distinctive Clinical Patterns

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FAILING SINGLE VENTRICLE PLASTIC BRONCHITIS Mechanism Similar to Protein-Losing Enteropathy

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FAILING SINGLE VENTRICLE PLASTIC BRONCHITIS TREATMENT • • • •

Inhaled steroids Albuterol Acetylcysteine Pulmonary physiotherapy THE HEART INSTITUTE FOR CHILDREN

FAILING SINGLE VENTRICLE PULMONARY VEINS COMPRESSION • External conduit • Leftward bulging of interatrial septum

THE HEART INSTITUTE FOR CHILDREN

CONCLUSIONS • Single ventricle failure is multifactorial.

• Aggressive early management is essential.

• Preoperative and intraoperative management can effect favorably the functional life of single ventricles.

THE HEART INSTITUTE FOR CHILDREN