Patient handout

Adult Fontan circulation (single-ventricle palliation, chronic ACHD)

PRODUCTION

1. Your condition

This handout is for adult fontan circulation (single-ventricle palliation, chronic achd). Your care team identified this based on: edema / ascites / declining exercise tolerance in fontan patient.

Other reasons your team may use this plan: iart / atrial flutter / af (poorly tolerated in fontan); chronic diarrhea / hypoalbuminemia / edema (protein-losing enteropathy); expectoration of bronchial casts (plastic bronchitis).

2. Your medications

Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.

Medication	Starting dose	How	When	What it does
euvolemia + low-PVR maintenance + relieve Fontan-pathway obstruction (stent) + maintain sinus rhythm	—	—	—	Fontan output is preload-dependent + PVR-sensitive — avoid dehydration, treat pathway stenosis, preserve sinus rhythm (2019 AHA Fontan statement)

Plan: Fontan — hemodynamic optimisation + failing-domain-directed therapy (2018 AHA/ACC ACHD; 2020 ESC ACHD; 2019 AHA Fontan statement)

3. When to call your provider

Contact your care team if any of the following happen:

Poorly-tolerated IART/AF → urgent rhythm control + AC + ablation — 2020 ESC ACHD
Refractory PLE/plastic bronchitis / failing Fontan → transplant evaluation — 2019 AHA Fontan statement
HCC on surveillance → hepatology/oncology + transplant pathway — 2019 AHA Fontan statement

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

IART/atrial flutter/AF in Fontan — poorly tolerated (loss of atrial kick + passive flow); urgent rhythm control + anticoagulation + ablation; pacing for sinus-node dysfunction — 2020 ESC ACHD(life-threatening)
Fontan-associated liver disease — serial elastography + HCC ultrasound (±AFP); cirrhotic FALD informs combined heart-liver transplant — 2019 AHA Fontan statement
Protein-losing enteropathy (hypoalbuminemia, edema, diarrhea, ↑stool α1-AT) — multimodal: high-protein diet, albumin, budesonide, anticoagulation, fenestration, transplant — 2019 AHA Fontan statement
Plastic bronchitis (bronchial casts) — lymphatic imaging + thoracic-duct/selective lymphatic embolization + Fontan optimisation — 2019 AHA Fontan statement
Fontan thromboembolism (pathway thrombus / stroke / PE) or high-risk — lifelong anticoagulation (warfarin; DOAC selected); aspirin lower-risk — 2019 AHA Fontan statement
Fontan-pathway stenosis/obstruction or elevated PVR — treatable failing-Fontan driver: catheter intervention/stent ± pulmonary vasodilator — 2020 ESC ACHD
Refractory failing Fontan (ventricular dysfunction / multiorgan failure) — transplant (heart, or combined heart-liver if advanced FALD) — 2019 AHA Fontan statement(life-threatening)
Pregnancy with Fontan — very high risk (mWHO III–IV): arrhythmia, thrombosis, PLE, ventricular failure, fetal loss; expert cardio-obstetric; many advised against pregnancy — ESC 2018 Pregnancy(life-threatening)

5. Follow-up

Lifelong Fontan-centre care; pregnancy very-high-risk counseling; transition

6. Sources

Guideline: 2018 AHA/ACC Adult Congenital Heart Disease Guideline (Stout) + 2020 ESC Adult Congenital Heart Disease Guideline (Baumgartner) + 2019 AHA Fontan Scientific Statement (Rychik)