Thrombocytopenia is Associated with Higher Fontan Pressure and Increased Morbidity in Patients with Fontan Circulation.

After the Fontan procedure, patients require lifelong follow-up due to significant late morbidity and mortality. Thrombocytopenia is seen frequently post-Fontan, likely due to secondary hypersplenism from elevated Fontan pressure. We investigated platelet counts in patients with a Fontan circulation and assessed associations with catheterization data and clinical outcomes. This retrospective study included 92 patients (33% female) post-Fontan who had a complete blood count performed between January 2011 and July 2023. The age at evaluation was 24.0 ± 8.9 years. Outcomes measured included elevated Fontan pressure (≥ 15 mmHg), Fontan-associated liver disease (FALD), unscheduled admissions, transplant, and death. Participants with thrombocytopenia (≤ 150,000/µL) had significantly higher rates of elevated Fontan pressure (OR 8.1, 95% CI 1.3-52.7, p = 0.03), FALD (OR 4.1, 95% CI 1.6-10.6, p = 0.004), and unscheduled admissions (362 ± 577 versus 115 ± 185 admissions per 1000 patient-years, p = 0.02). Thrombocytopenia post-Fontan is associated with elevated Fontan pressure, FALD, and increased morbidity. Platelet count could serve as a non-invasive factor in identifying patients at risk of decompensation.

Augmented Biphasic Breathing Using Sniff and an Oral Positive Expiratory Pressure Device (Sniff-PEP) in Fontan Patients.

The use of an oral positive expiratory pressure device (oPEP) with sniff breathing (Sniff-PEP) mimics biphasic ventilation. Biphasic ventilation increases pulmonary blood flow and cardiac output in Fontan patients. The aim of this study was to assess the effect of Sniff-PEP on Fontan flow velocities. A single-center, pilot, prospective study was carried out in 15 subjects with Fontan circulation enrolled to use the oPEP device for 1 month. Subjects were instructed on Sniff-PEP and to use the device for 10-15 min 3-4 times a day. Measurements of flow velocity and cardiac output were measured via echocardiogram and quality of life assessments were performed at baseline and 4-6 weeks later. The mean age at enrollment was 19.9 ± 8.7 years (age range of 10-37 years). 7 patients (47%) had dominant left ventricle and 8 (53%) had an open fenestration. There was a statistically significant increase in flow velocities in the hepatic vein from 27.5 ± 7.6 to 35.1 ± 11.3 cm/s (p = 0.003), left pulmonary artery from 51.6 ± 16.6 to 57.6 ± 21.1 cm/s (p = 0.01), and right pulmonary artery from 43.1 ± 14.2 to 45.8 ± 17.2 cm/s (p = 0.04). With chronic use, the mean fenestration gradient slightly decreased from 4.5 ± 1.6 to 4.1 ± 1.9 mmHg but the difference was not statistically significant (p = 0.14). oPEP device therapy increased flow velocity in several areas in the Fontan circulation with acute use. Further studies are needed to assess the effects long term.Clinical Trial Registration: URL: https://clinicaltrials.gov . Unique identifier: NCT03251742.

Multimodality Imaging in Ebstein Anomaly.

Ebstein anomaly is the most common form of tricuspid valve congenital anomalies. The tricuspid valve is abnormal with different degrees of displacement of the septal leaflet and abnormal rotation of the valve towards the right ventricular outflow tract. In severe forms, it results in significant tricuspid regurgitation and requires surgical repair. There is an increased interest in understanding the anatomy of the tricuspid valve in this lesion as the surgical repair has evolved with the invention and wide adoption of the cone operation. Multimodality imaging plays an important role in diagnosis, follow-up, surgical planning and post-operative care. This review provides anatomical tips for the cardiac imagers caring for patients with Ebstein anomaly and will help provide image-based personalized medicine.

Oral budesonide as a therapy for protein-losing enteropathy in patients having undergone Fontan palliation.

BACKGROUND: Protein-losing enteropathy is associated with Fontan palliation for single-ventricle physiology and has been difficult to treat. Limited data suggest the successful use of oral budesonide (Entocort, AstraZeneca) as a palliative measure. METHODS: We examine our single-institution retrospective experience in eight patients who were treated with this therapy. RESULTS: Median pretherapy albumin level was 1.7 g/dL (range 1.0-2.7 g/dL), 3 months after therapy was 3.1 g/dL (range 2-4.8 g/dL), and by the end of the first year was 3.4 g/dL (range 2.1-5.3 g/dL). All patients had at least a transient improvement, and at latest follow-up (median 29 months, range 3-51 months) five patients remain on therapy. Five of eight patients had required pretherapy albumin transfusions; one patient required albumin infusions after therapy. Four patients had side effects associated with the medication. CONCLUSIONS: Oral budesonide is an additional therapy that has the potential to improve symptoms and delay need for heart transplantation in this patient population.

Life threatening parvovirus B19 and herpes simplex virus associated acute myocardial dysfunction in a child with homozygous sickle cell disease.

Human parvovirus (HPV) B19, a common infection, frequently causes transient red cell aplasia in children with hemolytic anemia, such as sickle cell disease (SCD). It was considered to be a self-limited condition, easily treated with blood transfusion. However, acute splenic sequestration, acute chest syndrome, nephrotic syndrome, and stroke have been reported in SCD patients following HPV B19 infection. We report a 3-year-old child with SCD who developed fulminant myocarditis following HPV B19-related aplastic crisis. The diagnosis of myocarditis should be considered in a patient with hemolytic anemia with an infection with HPV B19 who develops signs of cardiopulmonary failure despite correction of anemia.

Elevation of tricuspid regurgitant jet velocity, a marker for pulmonary hypertension in children with sickle cell disease.

BACKGROUND: Pulmonary hypertension (PHTN) is a potentially life-threatening complication, detected by echocardiographic evidence of elevated tricuspid regurgitant velocity (TRV). This condition has been described in adults with sickle cell disease (SCD) and other hemolytic disorders; however, there is little information on the occurrence of this condition in pediatric patients. METHODS: Records for pediatric SCD patients were retrospectively reviewed to determine clinical characteristics and co-morbidities of patients with elevated TRV on echocardiograms obtained under steady state conditions as an outpatient. Correlation of TRV > or =2.5 m/sec with age, sex, type of SCD, number of outpatient echocardiograms per patient, episodes of vasoocclusive crisis (VOC) and acute chest syndrome (ACS), mean hemoglobin and reticulocyte count, asthma, obstructive sleep apnea, cerebrovascular disease (CVD), and hydroxyurea therapy was determined. RESULTS: Of 224 SCD patients, 44 had outpatient echocardiographic measurement of TRV. Patients (11 of 44) (26.2%) with TRV > or =2.5 m/sec were compared to 31 patients without elevated TRV. Significant differences were noted for percent with HbSS disease (P = 0.041), CVD (P = 0.021), hemoglobin (P = 0.003), % reticulocytes (P = 0.037), and number of echocardiograms performed (P < 0.001). No significant differences were observed for gender, age, asthma, or frequency of VOC and ACS. CONCLUSIONS: Elevated TRV, a surrogate marker for PHTN, occurs in children with SCD and is associated with low hemoglobin, elevated reticulocyte count, and cerebral vasculopathy. Appropriate screening by echocardiography can lead to detection and treatment that may reduce TRV and potentially reverse the disease process, prevent the increased morbidity and mortality associated with PHTN.