Hybrid stage 1 palliation as a bridge to cardiac transplantation in patients with high-risk single ventricle physiology

2018 ◽  
Vol 22 (8) ◽  
pp. e13307 ◽  
Author(s):  
Brian H. Morray ◽  
Erin L. Albers ◽  
Thomas K. Jones ◽  
Mariska S. Kemna ◽  
Lester C. Permut ◽  
...  
Keyword(s):  
High Risk ◽  
Cardiac Transplantation ◽  
Single Ventricle ◽  
Single Ventricle Physiology ◽  
Stage 1 ◽  
Hybrid Stage 1 Palliation

Abstract 12134: Risk of In-Hospital Deterioration for Children With Single Ventricle Physiology

Circulation ◽  
2021 ◽  
Vol 144 (Suppl_2) ◽  
Author(s):  
Henry Foote ◽  
Zohaib Shaikh ◽  
William Ratliff ◽  
Michael Gao ◽  
Bradley Hintze ◽  
...  
Keyword(s):  
High Risk ◽  
Pediatric Cardiology ◽  
Single Ventricle ◽  
Retrospective Chart Review ◽  
Clinical Deterioration ◽  
Inpatient Service ◽  
Lower Systolic Blood Pressure ◽  
Single Ventricle Physiology ◽  
Entire Cohort ◽  
Increased Risk

Introduction: Children with single ventricle physiology (SV) are at high risk of in-hospital morbidity and mortality, with much of that increased risk coming in the first year of life. Understanding which children are at the highest risk for clinical deterioration may allow for increased monitoring and earlier escalation of care, with associated decreased mortality. Methods: We conducted a retrospective chart review of all admissions to the pediatric cardiology non-ICU inpatient service from 2014 - 2018 for children < 18 years old. Clinical deterioration was defined as an unplanned transfer to the ICU or inpatient mortality. Children with SV were selected by diagnosis codes. Results: From the entire cohort of 1612 pediatric cardiology admissions (56 % male, 25% SV), 288 admissions had a deterioration event including 26 deaths. Infants less than one year with SV (n = 197 admissions) were significantly more likely to have a deterioration event (107 events over 62 admissions with an event) than the overall pediatric cardiology cohort (OR 2.11, 95% CI 1.52-2.93). Among infants with SV, those with a deterioration event were significantly younger (median 1.7 v 4.3 months, p < 0.001). Further, at baseline they had significantly lower oxygen saturation (84% v 87%, p < 0.01), lower systolic blood pressure (85mmHg v 90mmHg, p< 0.02), higher respiration rate (48 v 44, p < 0.01), and higher hematocrit (44.0 v 40.2, p < 0.005) compared to those who remained stable. Mean Pediatric Early Warning Scores (PEWS) were significantly higher for infants with SV who had a deterioration event (1.4 v 0.9, p < 0.001) and PEWS scores significantly increased in the 48 hours prior to an event (p < 0.001). Of the 104 non-death events, 61 required an increase in oxygen support and 51 required a fluid bolus prior to the event (p < 0.001). Conclusions: Infants with SV are at high risk for clinical deterioration. There are baseline differences in vital signs and lab work between those that remain stable and those that have a deterioration event. PEWS scores and oxygen and fluid treatment significantly increase prior to deterioration events. Leveraging data from the Electronic Medical Record to identify the highest risk patients may allow for earlier detection and intervention to prevent clinical deterioration.

scholarly journals Pulmonary Vasodilator Therapy in Children with Single Ventricle Physiology: Effects on Saturation and Pulmonary Arterial Pressure

2020 ◽  
Vol 41 (8) ◽  
pp. 1651-1659
Author(s):  
Ida Jeremiasen ◽  
Karin Tran-Lundmark ◽  
Nikmah Idris ◽  
Phan-Kiet Tran ◽  
Shahin Moledina
Keyword(s):  
Arterial Pressure ◽  
Single Ventricle ◽  
Pulmonary Arterial Pressure ◽  
Vasodilator Therapy ◽  
Single Ventricle Physiology ◽  
Pulmonary Vasodilators ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Vasodilator ◽  
Pulmonary Arterial ◽  
Stage 1

AbstractIn children with single ventricle physiology, increased pulmonary vascular resistance may impede surgical progression or result in failing single ventricle physiology. The use of pulmonary vasodilators has been suggested as a potential therapy. However, knowledge on indication, dosage, and effect is limited. A retrospective case notes review of all (n = 36) children with single ventricle physiology, treated with pulmonary vasodilators by the UK Pulmonary Hypertension Service for Children 2004–2017. Therapy was initiated in Stage 1 (n = 12), Glenn (n = 8), or TCPC (n = 16). Treatment indications were high mean pulmonary arterial pressure, cyanosis, reduced exercise tolerance, protein-losing enteropathy, ascites, or plastic bronchitis. Average dose of sildenafil was 2.0 mg/kg/day and bosentan was 3.3 mg/kg/day. 56% had combination therapy. Therapy was associated with a reduction of the mean pulmonary arterial pressure from 19 to 14 mmHg (n = 17, p < 0.01). Initial therapy with one or two vasodilators was associated with an increase in the mean saturation from 80 to 85%, (n = 16, p < 0.01). Adding a second vasodilator did not give significant additional effect. 5 of 12 patients progressed from Stage 1 to Glenn, Kawashima, or TCPC, and 2 of 8 from Glenn to TCPC during a mean follow-up time of 4.7 years (0–12.8). Bosentan was discontinued in 57% and sildenafil in 14% of treated patients and saturations remained stable. Pulmonary vasodilator therapy was well tolerated and associated with improvements in saturation and mean pulmonary arterial pressure in children with single ventricle physiology. It appears safe to discontinue when no clear benefit is observed.

Severe airway obstruction from a bronchial cast after cardiac transplantation

2013 ◽  
Vol 24 (4) ◽  
pp. 739-740 ◽  
Author(s):  
John J. Parent ◽  
Robert K. Darragh
Keyword(s):  
Congenital Heart Disease ◽  
Case Report ◽  
Cardiac Transplantation ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Disease Process ◽  
Plastic Bronchitis ◽  
Single Ventricle Physiology ◽  
Bronchial Cast ◽  
Severe Airway Obstruction

AbstractPlastic bronchitis is a rare and difficult to treat disease process in patients with congenital heart disease. Cardiac transplantation has been used increasingly to reverse this process, especially in single ventricle physiology. This case report demonstrates a foreseeable complication after cardiac transplantation in such a patient.

scholarly journals Which criteria should be used to select patients for the Fontan operation?

2020 ◽  
Author(s):  
Melvin C Almodovar ◽  
Leonardo Mulinari
Keyword(s):  
High Risk ◽  
Single Ventricle ◽  
Fontan Operation ◽  
Single Ventricle Physiology ◽  
Pulmonary Pressure ◽  
Borderline Cases ◽  
Pulmonary Flow ◽  
Risk Patients ◽  
Fontan Palliation ◽  
Flow Study

The Fontan operation has improved the survival of children born with single ventricle physiology. Selecting candidates for the Fontan operation may be difficult on borderline cases. No clear criterion has been established on the risk for staged Fontan palliation. Another aspect that remains controversial is the indications for fenestration. Intraoperative pulmonary flow study may identify high-risk patients for the procedure. In this report, the authors describe their results with Fontan procedures in children with pulmonary pressure >15 mmHg.

scholarly journals CRT Implantation in a Patient with Congenital Heart Malformation

2019 ◽  
Vol 46 (3) ◽  
pp. 44-45
Author(s):  
S. Iovev ◽  
N. Chilingirova ◽  
B. Atzev
Keyword(s):  
High Risk ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Fontan Procedure ◽  
Functional Class ◽  
Single Ventricle Physiology ◽  
Heart Malformation ◽  
Surgical Corrections ◽  
Ventricle Function

Abstract Treatment of patients with functional single-ventricle physiology is achieved using surgical corrections known as the “Fontan Procedure”. Two separate blood circulations are created from the single-ventricle function. These are non-cyanogenic patients, but at high risk of developing HF. CRT implantation is an option to improve the functional class and EF.

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