Long-term Issues After the Fontan Procedure

McRae, Marion E.

doi:10.1097/nci.0b013e31829744c7

Cited by 18 publications

(25 citation statements)

References 74 publications

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“…The 2014 HRC/PACES guidelines recommend implantable cardioverter defibrillator (ICD) implantation in ACHD patients with poor systemic ventricular function, following hemodynamically significant ventricular arrhythmias and in selected higher‐risk patients with TOF. Unfortunately, transvenous ICD implantation can be challenging in the ACHD population, as a result of their complex anatomy and associated risks of procedural complications . Particular difficulty exists following the Fontan procedure for single ventricle, which often precludes access from the venous system to the heart .…”

Section: Introductionmentioning

confidence: 99%

“…The most recent development in ICD design is the subcutaneous ICD (S‐ICD) system, which comprises a generator placed in the anterolateral position, and a left parasternal coil placement. The absence of transvenous access preserves the vasculature and is thought to minimize procedure‐related complications, risk of bleeding, thrombosis, and infection . The S‐ICD is an attractive option in ACHD patients who are likely to require multiple generator changes in their lifetime and face potential complications with each intervention, particularly when transvenous access to the ventricles is difficult.…”

Section: Introductionmentioning

confidence: 99%

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Eligibility for subcutaneous implantable cardioverter defibrillators in the adult congenital heart disease population

Garside

Leyva

Hudsmith

et al. 2018

Pacing Clinical Electrophis

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Background Patients with adult congenital heart disease (ACHD) have an increased risk of arrhythmic and sudden cardiac death. The subcutaneous implantable cardioverter defibrillator (S‐ICD) provides a potentially safer alternative to transvenous ICDs in ACHD. Suitability for S‐ICD depends on the surface electrocardiogram (ECG), which is often abnormal in ACHD patients. This study investigates the proportion of ACHD patients who meet the screening criteria for S‐ICD implantation. Methods A standard screening ECG was performed in 102 patients with complex ACHD (Tetralogy of Fallot, Fontan circulation, and transposition of the great arteries). This process was repeated postexercise for patients who also had an exercise test. Results Three quarters (75.4%) of ACHD patients meet screening criteria for an S‐ICD with at least one suitable vector. The most common number of acceptable vectors in the eligible group was two (35% of total population). In only 12% of total population, all three vectors were suitable while only one vector was suitable in 28% of total population. The primary vector (equivalent of ECG lead III) was the most common suitable vector, found in 62% of participants who had appropriate sensing vectors. Twenty‐five (24.5%) patients failed to meet the S‐ICD screening criteria. Of these, 14 had repaired tetralogy of Fallot. A total of 92% of patients with a Fontan circulation met ECG screening criteria. Of those who had the protocol repeated following their cardiopulmonary exercise test (n = 14), only one additional patient failed to meet the eligibility criteria. Conclusions A quarter of ACHD patients do not meet the eligibility criteria for the S‐ICD. However, more than 90% of patients with a Fontan circulation are suitable for an S‐ICD.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Eligibility for subcutaneous implantable cardioverter defibrillators in the adult congenital heart disease population

Garside

Leyva

Hudsmith

et al. 2018

Pacing Clinical Electrophis

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“…Counselling related to pregnancy should be started very early after surgery, and initially parents should be informed about the need for regular followup, the risks of pregnancy, and the need for prepregnancy planning, as certain drugs such as angiotensin converting inhibitors often used in Fontan patients need to be stopped before conception. 9 As these patients approach childbearing age, they should be counselled about contraception, the risk of pregnancy with Fontan circulation, and the need for thorough planning and monitoring if pregnancy is desired. Each Fontan patient should have detailed pre-conception counselling regarding the high miscarriage rates with expected psychological consequences, and the frequent occurrence of other clinically significant complications during pregnancy, especially maternal haemodynamic disturbances, the high incidence of premature labour and delivery, as well as the risks of neonatal death.…”

Section: Neonatal Complicationsmentioning

confidence: 99%

Twin pregnancy in a Fontan-palliated patient

2016

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The Fontan connection, originally described in 1971, is used to provide palliation for patients with many forms of CHDs that cannot support a biventricular circulation. An increasing number of females who have undergone these connections in childhood are now surviving into adulthood and some are becoming pregnant. We report a case of a 29-year-old woman who presented with a twin pregnancy at 33 weeks of gestation. She had significant deterioration of her cardiovascular status before the twin babies were delivered by emergency caesarean section owing to associated obstetric complications. This report also highlights the various maternal and fetal complications occurring in pregnancy of Fontan-palliated patients and suggests the need for meticulous pre-conception counselling and strict perinatal care.

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“…[ 2 ] Despite saving numerous lives for the past 45 years, the Fontan circulation is often associated with long-term complications such as heart failure, pleural effusion, hypercoagulability, portal hypertension, and atrial fibrillation. [ 3 ] Among these, long-term elevated systemic venous pressure and low cardiac output may lead to the development of liver cirrhosis, and such a progression may eventually result in the incidence of hepatocellular carcinoma (HCC) in some patients. [ 4 ]…”

Section: Introductionmentioning

confidence: 99%

Hepatocellular carcinoma after the Fontan procedure in a 16-year-old girl

Youn²,

Han

et al. 2016

Medicine

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Introduction:The Fontan procedure (FP) has become the standard operation for patients with single ventricle physiology. However, a long period of elevated systemic venous pressure and low cardiac output after the procedure result in chronic inflammation and liver cirrhosis, which may eventually lead to the occurrence of hepatocellular carcinoma (HCC).Clinical Findings:We described the case of a 16-year-old female who developed HCC after the FP. At 21 months, the patient received a lateral tunnel FP, and 14 years later, she began complaining of abdominal distension, telangiectasia, and fatigue. Imaging studies revealed a large hepatic mass involving most of the right lobe and multiple masses in the left lobe. Evidence suggested severe liver cirrhosis, and the presence of ascites, hepatosplenomegaly, paraesophageal gastric varices reflecting the severity of the disease. In addition, tumor thrombosis was found in the right hepatic vein, middle hepatic vein, and inferior vena cava, as well as multiple metastatic nodules in both lungs. The patient received an incisional biopsy and the diagnosis of HCC was pathologically confirmed. After treatment with 1 cycle of systemic chemotherapy, she received ongoing supportive care for disease-related complications, and died 2 months after chemotherapy due to hematemesis.Conclusion:With the advances in medicine, the incidence of Fontan physiology-related complications is likely to increase, and the incidence of HCC will also increase accordingly. As early diagnosis of HCC results in better patient outcomes, a surveillance guideline for HCC after the FP should be developed.

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Long-term Issues After the Fontan Procedure

Cited by 18 publications

References 74 publications

Eligibility for subcutaneous implantable cardioverter defibrillators in the adult congenital heart disease population

Eligibility for subcutaneous implantable cardioverter defibrillators in the adult congenital heart disease population

Twin pregnancy in a Fontan-palliated patient

Hepatocellular carcinoma after the Fontan procedure in a 16-year-old girl

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