Understanding the Fontan Procedure in Congenital Heart Disease Patients and the Importance of Early Hepatology Referrals: A Case Study

2018 ◽  
Vol 11 (2) ◽  
pp. 95-99
Author(s):  
Alice Chan
Keyword(s):  
Primary Care ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Nurse Practitioners ◽  
Congenital Heart ◽  
Care Setting ◽  
Primary Care Setting ◽  
Fontan Procedure ◽  
Early Screening

Background: Patients with congenital heart disease are surviving into adulthood because of new surgeries developed over the recent decades. One surgery is the Fontan procedure, which is used in patients with only one functioning ventricle. Although it has been saving lives, in the recent years multiple complications have been noted. Objective: To discuss the Fontan procedure and inform nurse practitioners in the primary care setting the importance of early screening and referral because of increasing number of liver complications in these patients. This case study highlights the dilemmas a patient with the Fontan procedure faces as a young adult. Methods: Evidence-based articles from the PubMed and Embase databases were used to support the case study. Results: Patients with the low-pressure Fontan circulation are under chronic passive congestion and have known liver complications such as liver fibrosis, cirrhosis, and protein-losing enteropathy. This can lead to a high-risk dual organ heart–liver transplant. Conclusions: Early screening and referral to a hepatologist are critical in these patients. Implications for Nursing: Since patients with congenital heart disease often present to the primary care setting, nurse practitioners in this area should understand the Fontan procedure and its associated complications so they can effectively manage these patients.

scholarly journals Evaluation of an unscheduled care model delivered by advanced nurse practitioners in a primary-care setting

2019 ◽  
Vol 24 (8) ◽  
pp. 696-709
Author(s):  
Calum F Leask ◽  
Heather Tennant
Keyword(s):  
Primary Care ◽  
Nurse Practitioners ◽  
Care Setting ◽  
Primary Care Setting ◽  
Home Visits ◽  
Rejection Rate ◽  
Service Level ◽  
Average Score ◽  
Unscheduled Care ◽  
Viable Solution

Background Considering new models of delivery may help reduce increasing pressures on primary care. One potentially viable solution is utilising Advanced Practitioners to deliver unscheduled afternoon visits otherwise undertaken by a General Practitioner (GP). Aims Evaluate the feasibility of utilising an Advanced Nurse Practitioner (ANP) to deliver unscheduled home visits on behalf of GPs in a primary care setting. Methods Following a telephone request from patients, ANPs conducted unscheduled home visits on behalf of GPs over a six-month period. Service-level data collected included patient-facing time and outcome of visits. Practice staff and ANPs participated in mind-mapping sessions to explore perceptions of the service. Results There were 239 accepted referrals (total visiting time 106.55 hours). The most common outcomes for visits were ‘medication and worsening statement given’ (107 cases) and ‘self-care advice’ (47 cases). GPs were very satisfied with the service (average score 90%), reporting reductions in stress and capacity improvements. Given the low referral rejection rate, ANPs discussed the potential to increase the number of practices able to access this model, in addition to the possibility of utilising other practitioners (such as paramedics or physiotherapists) to deliver the same service. Conclusions It appears delivering unscheduled care provision using an ANP is feasible and acceptable to GPs.

scholarly journals An unusual cause of edema in a child with congenital heart disease post Fontan procedure

2019 ◽  
Vol 7 (11) ◽  
pp. 4396
Author(s):  
Minal Wade ◽  
Shweta Shettiwar ◽  
Ankita Shah
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Rare Complication ◽  
Fontan Procedure ◽  
Protein Losing Enteropathy ◽  
Common Causes ◽  
Disease Protein ◽  
The Common ◽  
Fontan Palliation

Protein Losing Enteropathy Post Fontan procedure. Protein Losing Enteropathy (PLE) is an uncommon cause of edema in children with congenital heart disease. Protein-Losing Enteropathy may be defined as excessive loss of proteins across the intestinal mucosa and is due to either a primary gastrointestinal abnormality or secondary to cardiac disease. Protein-losing enteropathy (PLE) is a rare complication of the Fontan palliation for functional single-ventricle. Although PLE occurs in about 3.5% of patients post-Fontan, it confers marked morbidity and high mortality within 5 years of diagnosis. The pathogenesis of Fontan-related PLE is not completely understood, and it is unclear why it develops in some patients post-Fontan and not others. We describe a child with Double Inlet Right Ventricle who had undergone Fontan procedure, and presented to us with generalised oedema. The child had hypoproteinaemia, the common causes for which were ruled out and was diagnosed as Protein Losing Enteropathy (PLE) related to his surgical intervention. Though, not frequently encountered it should be kept in mind as one of the causes of anasarca.

Chylothorax after surgery for congenital heart disease in children

1998 ◽  
Vol 8 (2) ◽  
pp. 199-204 ◽  
Author(s):  
Simone Rolim Fernandes Fontes Pedra ◽  
Carlos Augusto Cardoso Pedra ◽  
Valmir Fernandes Fontes ◽  
Maria Virgínia Tavares Santana ◽  
Paulo Paredes Paulista
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Fontan Procedure ◽  
Bidirectional Glenn ◽  
Surgical Data ◽  
Considerable Morbidity ◽  
Classical Means ◽  
Hypercaloric Diet ◽  
High Output

AbstractWe reviewed retrospectively the clinical and surgical data from 9 children with postoperative chylothorax secondary to cardiac surgery for congenital heart disease seen over a 3 year period. Mean age was 26 months and 6 patients were female. The procedures performed were 2 Blalock-Taussig shunts, 6 Bidirectional Glenn anastomoses and 1 modified Fontan procedure. Diagnosis of chylothorax was made by classical means. The overall incidence of chylothorax was 0.8%, with the relative incidences being 2.5% for Blalock-Taussig, 2.7% for Fontan, and 11.7% for the Glenn procedures. All patients were initially treated conservatively with chest tube drainage and hypercaloric diet suplemented with medium chain triglycerides. Parenteral nutrition was needed in 2 patients, one because of sepsis and the other because of progressive malnourishment. High output fistulas and long periods of drainage were noted after cavo-pulmonary procedures. Up to 12% weight loss was observed in 6 patients, one patient was successfully treated of pneumonia and another died ofPseudomonas aeruginosasepsis. Two patients required chemical pleurodesis because of unabated drainage, with the others being successfully treated conservatively. Chylothorax is a common complication after bidirectional Glenn procedures, and is associated with considerable morbidity and mortality. Earlier operative intervention may be required in these patients. Pleurodesis is a simple, safe, and effective procedure to stop chylous flow.

scholarly journals Pulmonary arterial hypertension populations of special interest: portopulmonary hypertension and pulmonary arterial hypertension associated with congenital heart disease

2019 ◽  
Vol 21 (Supplement_K) ◽  
pp. K37-K45 ◽  
Author(s):  
Laurent Savale ◽  
Alessandra Manes
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Congenital Heart ◽  
Expert Advice ◽  
Portopulmonary Hypertension ◽  
Early Screening ◽  
Pulmonary Arterial

Abstract Guidelines exist for management of pulmonary arterial hypertension (PAH), but information is limited for certain patient subgroups, including adults with portopulmonary hypertension (PoPH) or with PAH associated with congenital heart disease (PAH-CHD). This article discusses screening, clinical management, and prognosis in PoPH and PAH-CHD and, as such, considers the most recent clinical data and expert advice. A multidisciplinary consultation and follow-up by specialists are crucial for management of both PoPH and PAH-CHD, but each condition presents with unique challenges. Development of PoPH most commonly occurs among patients with liver cirrhosis. Initially, patients may be asymptomatic for PoPH and, if untreated, survival with PoPH is generally worse than with idiopathic PAH (IPAH), so early identification with screening is crucial. PoPH can be managed with PAH-specific pharmacological therapy, and resolution is possible in some patients with liver transplantation. With PAH-CHD, survival rates are typically higher than with IPAH but vary across the four subtypes: Eisenmenger syndrome, systemic-to-pulmonary shunts, small cardiac defects, and corrected defects. Screening is also crucial and, in patients who undergo correction of CHD, the presence of PAH should be assessed immediately after repair and throughout their long-term follow-up, with frequency of assessments determined by the patient’s characteristics at the time of correction. Early screening for PAH in patients with portal hypertension or CHD, and multidisciplinary management of PoPH or PAH-CHD are important for the best patient outcomes.

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