78th Annual PostGraduate Assembly in Anesthesiology (PGA78) 2024

Background:

Double Outlet Right Ventricle (DORV):

•1/10,000 live births1

•Features:2 (Figure 1)

oPulmonary artery and aorta arise from the right ventricle

oConcomitant VSD

oHypoplastic left ventricle

•Location of the VSD, presence of pulmonary stenosis, and other concomitant cardiac abnormalities determine hemodynamics and palliation strategy of the DORV

Procedural interventions:3

•Biventricular repair

•Single ventricle palliation

oGlenn, Fontan

Long term complications of single ventricle palliation:4

•Arrythmia

•Protein-losing enteropathy

•Atrioventricular valve insufficiency

•Plastic bronchitis

•Conduit stenosis

Case description:

37-year-old female underwent Fontan angiography and conduit stenting

Past Medical History:

•DORV, unbalanced AV canal defect

os/p bilateral bidirectional Glenn (1993)

os/p extracardiac Fontan procedure (1996) (Figure 3)

os/p mechanical atrioventricular valve (2005) on warfarin

•Multi-system manifestations: liver cirrhosis, right MCA stroke

Intraoperative course:

•Deep sedation (propofol and dexmedetomidine infusions), spontaneously breathing (SpO2 97%)

•Spontaneous pulmonary hemorrhage after endovascular stenting and balloon dilation of Fontan conduit

Challenges and management strategies:

Postoperative complications:

•ARDS secondary to pneumonia - initiation of VV ECMO (POD 4)

•Subarachnoid hemorrhage, no recurrent pulmonary hemorrhage

Discussion:

•Spontaneous respirations is most physiologically favorable due to maintaining negative intrathoracic pressure.

•Patients with Fontan circulation are more prone to pulmonary hemorrhage given increased pulmonary collaterals5 and the use of anticoagulants.

•Lung isolation techniques may be needed for lung protection but the resulting shunt can increase pulmonary vascular resistance.

•Acute hypovolemia and increased pulmonary vascular resistance can rapidly precipitate hypoxia, hypercarbia, decreased cardiac output, ischemia of the single ventricle and ultimately lead to cardiac arrest.6

•Resuscitation efforts should focus on inotropic support of single ventricle, preload maintenance, and PVR reduction.

•Early consideration of ECMO; limited vascular access, and prior repeated sternotomies make initiation of ECMO challenging.

78th Annual PostGraduate Assembly in Anesthesiology (PGA78) 2024

Management of a Pulmonary Hemorrhage During Percutaneous Fontan Conduit Stenting

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