Myocardial Work by Speckle-Tracking Echocardiography in Heart Transplant Recipients: Association Between Global Work Efficiency and Coronary Allograft Vasculopathy.

Coronary allograft vasculopathy (CAV) is a leading cause of morbidity and mortality after heart transplantation. CAV is often diagnosed in later stages or during routine screening in asymptomatic subjects. Myocardial work (MW), calculated using left ventricular global longitudinal strain (LV-GLS) and systemic blood pressure, may be associated with the presence of CAV and outperform conventional echocardiographic parameters. In this retrospective observational study, heart transplant recipients who underwent regular follow-up at our institution between May 2022 and September 2023 were enrolled. All included patients underwent speckle-tracking echocardiography, including MW indexes. CAV was classified according to invasive coronary angiography or computed tomography performed within 12 months of index echocardiography. We collected all available clinical and echocardiographic parameters and evaluated the potential association with CAV. CAV was detected in 29 of 93 patients (31%) (CAV+). Of the MW indexes, the mean global work efficiency (GWE) was 90 ± 6% and was significantly lower in CAV+ than CAV- subjects (86 ± 7% vs 91 ± 4%, p <0.001). GWE (OR 0.86, CI 0.77 to 0.94, p = 0.002), E/e' ratio (OR 1.27, CI 1.08 to 1.52, p = 0.006), and left ventricular ejection fraction (OR 0.90; CI 0.81 to 0.98, p = 0.017) were independently associated with the presence of CAV. GWE (GWE vs LV-GLS, delta area under the curve 0.154, p = 0.047) and the proposed model (GWE+E/e' vs LV-GLS, delta area under the curve 0.198, p = 0.004) were significantly superior in stratifying the incremental risk for CAV compared with LV-GLS. In conclusion, GWE was observed to be independently associated with the presence of CAV. MW could represent a novel noninvasive screening method for CAV in heart transplant recipients. Larger and prospective studies are needed to confirm this hypothesis.

Computed tomography pericoronary adipose tissue density predicts coronary allograft vasculopathy and adverse clinical outcomes after cardiac transplantation.

AIMS: To assess pericoronary adipose tissue (PCAT) density on coronary computed tomography angiography (CCTA) as a marker of inflammatory disease activity in coronary allograft vasculopathy (CAV). METHODS AND RESULTS: PCAT density, lesion volumes, and total vessel volume-to-myocardial mass ratio (V/M) were retrospectively measured in 126 CCTAs from 94 heart transplant patients [mean age 49 (SD 14.5) years, 40% female] who underwent imaging between 2010 and 2021; age- and sex-matched controls; and patients with atherosclerosis. PCAT density was higher in transplant patients with CAV [n = 40; -73.0 HU (SD 9.3)] than without CAV [n = 86; -77.9 HU (SD 8.2)], and controls [n = 12; -86.2 HU (SD 5.4)], P < 0.01 for both. Unlike patients with atherosclerotic coronary artery disease (n = 32), CAV lesions were predominantly non-calcified and comprised of mostly fibrous or fibrofatty tissue. V/M was lower in patients with CAV than without [32.4 mm3/g (SD 9.7) vs. 41.4 mm3/g (SD 12.3), P < 0.0001]. PCAT density and V/M improved the ability to predict CAV from area under the receiver operating characteristic curve (AUC) 0.75-0.85 when added to donor age and donor hypertension status (P < 0.0001). PCAT density above -66 HU was associated with a greater incidence of all-cause mortality {odds ratio [OR] 18.0 [95% confidence interval (CI) 3.25-99.6], P < 0.01} and the composite endpoint of death, CAV progression, acute rejection, and coronary revascularization [OR 7.47 (95% CI 1.8-31.6), P = 0.01] over 5.3 (SD 2.1) years. CONCLUSION: Heart transplant patients with CAV have higher PCAT density and lower V/M than those without. Increased PCAT density is associated with adverse clinical outcomes. These CCTA metrics could be useful for the diagnosis and monitoring of CAV severity.

Elevated Aortic Stiffness after Pediatric Heart Transplantation.

In adults, arterial stiffness has been linked to the development of target end-organ damage, thought to be related to abnormal transmission of pulse pressure. Increased arterial stiffness and endothelial dysfunction have been hypothesized to contribute to the development of microvascular dysfunction and coronary allograft vasculopathy (CAV), an important comorbidity after heart transplantation. However, little data exists regarding arterial stiffness in pediatric heart transplantation and its influence on development of coronary allograft vasculopathy is not well understood. We sought to assess aortic stiffness and distensibility in pediatric post-heart transplant patients. A prospective, observational study analyzing the ascending (donor tissue) and descending aorta (recipient tissue) using transthoracic echocardiographic M-mode measurements in patients aged < 21 years was conducted. Descending and ascending aorta M-modes were obtained from the subcostal long axis view, and the parasternal long axis view 3-5mm above the sinotubular junction, respectively. Two independent reviewers averaged measurements over 2-3 cardiac cycles, and Aortic Distensibility (AD) and Aortic Stiffness Index (ASI) were calculated using previously validated methods. We recruited 39 heart transplant (HT) patients and 47 healthy controls. Median end diastolic dimension of the ascending aorta (donor tissue) was significantly larger in the transplant group than the control group (1.92 cm vs. 1.74 cm, p = 0.01). Ascending aortic distensibility in post-transplant patients was significantly lower than in the control group (4.87 vs. 10.53, p < 0.001). Ascending aortic stiffness index was higher in the transplant patients compared to the controls (4.63 vs. 2.21, p < 0.001). There is evidence of altered ascending aortic distensibility and stiffness parameters in post-heart transplant patients. Further studies are required to assess its influence on complications like development of coronary artery vasculopathy.

The risks of rejection vs. infection: Ramsay Hunt syndrome, Gradenigo syndrome, and varicella meningoencephalitis in a heart transplant patient.

Background: Orthotopic heart transplant (OHT) recipients are at increased risk for varicella zoster reactivation, and severe complications may arise due to their immunosuppressive regimens. Managing immunosuppression in acute infection is difficult, and specific guideline recommendations or evidence from the literature are lacking. However, patient care must involve weighing the risk of transplant rejection with the consequences of worsening infection. Case summary: An OHT patient with a history of multiple episodes of acute rejection, latent varicella zoster virus (VZV) infection, and recent completion of anti-viral prophylaxis presented with unilateral facial droop and pain, abducens nerve palsy, crusting facial rash, and ear swelling. Imaging revealed necrotizing otitis externa, with associated otitis media, and petrous apicitis concerning for Gradenigo syndrome. A VZV-positive viral panel confirmed our suspicion for Ramsay Hunt syndrome (RHS). The patient's mentation continued to decline, and subsequent lumbar puncture also revealed VZV meningoencephalitis. The patient's mycophenolate mofetil (MMF) was suspended, with continuation of tacrolimus, and initiation of intravenous acyclovir. The patient demonstrated gradual resolution of his infection, without developing any signs of acute rejection. Discussion: Varicella zoster virus reactivation is common in OHT patients, particularly when viral prophylaxis is discontinued; however, cardiologists should be aware of the rarer manifestations that can manifest in these immunocompromised patients. This is the first documented case of simultaneous RHS, Gradenigo syndrome, and VZV meningoencephalitis in any patient, regardless of transplant status. We demonstrate that even in patients at very high risk of rejection, MMF can be safely discontinued and host immunity maintained with temporary tacrolimus monotherapy.

Coronary allograft vasculopathy in post-heart transplant patients: pathogenesis and role of cardiac computed tomography in diagnosis-a comprehensive review.

Coronary allograft vasculopathy, often known as cardiac allograft vasculopathy (CAV), is a substantial source of morbidity and mortality in people who have had heart transplants. Early detection and monitoring of CAV are crucial for improving outcomes in this population. Although cardiac computed tomography (CT) has emerged as a possible method for finding and evaluating CAV, invasive coronary angiography has long been thought of as the gold standard for recognizing CAV. This study focuses on the utility of cardiac CT for CAV diagnosis and treatment in the post-heart transplant population. It provides an overview of recent studies on the application of cardiac CT in CAV and highlights the advantages and disadvantages of this imaging modality. The potential application of cardiac CT for CAV risk assessment and care is also examined in the study. Overall, the data point to a potential role for cardiac CT in the detection and treatment of CAV in post-heart transplant patients. It enables evaluation of the whole coronary tree and low-radiation, high-resolution imaging of the coronary arteries. Hence, further study is required to determine how best to employ cardiac CT in treating CAV in this group.

Clinical Value of Positron Emission Tomography Myocardial Perfusion Imaging and Blood Flow Quantification.

Myocardial blood flow (MBF) and flow reserve (MFR) measurements by PET/computed tomography provide incremental diagnostic and prognostic information over traditional quantification of ischemia and scar by myocardial perfusion imaging. A normal stress MBF and MFR (>2.0) have a very high negative predictive value for excluding high-risk obstructive coronary artery disease (CAD). These flow measurements are also used for surveillance of coronary allograft vasculopathy after heart transplantation. A global normal MFR (>2.0) identifies patients at lower clinical risk, whereas a severely reduced MFR (<1.5) identifies patients at high risk for adverse events, even among patients without regional perfusion abnormalities.

Increased fibrosis and microvessel disease in allograft endomyocardial biopsies of children with chronic graft failure due to cardiac allograft vasculopathy.

INTRODUCTION: Chronic graft failure (CGF) is the leading cause of mortality in pediatric heart transplant (PHT) patients and has multifactorial pathogenesis including cardiac allograft vasculopathy (CAV). CGF can present with microvessel disease (MVD) and myocardial fibrosis on endomyocardial biopsies (EMB). We investigated if CGF due to moderate- severe (M-S) CAV has histopathologic MVD and fibrosis prior to or at the time of CAV diagnosis. METHOD: This retrospective case-control study included PHT with CGF secondary to M-S CAV. Control patients had no CAV or CGF. EMBs from CAV (3 sets: at 1-year post-transplant 1yrCAV, pre-CAV, and at the time of CAV diagnosis) and non-CAV cohorts were reviewed to grade the fibrosis and quantify MVD. Histopathologic changes were correlated and compared between CAV/non-CAV groups. RESULTS: Each group had 8 patients. The median age at transplantation and time since transplant were similar between the two groups (P=.71 and P=.91, respectively). Fibrosis grade was 3.0 for CAV cohort compared to 1.0 for control (P= .003) and MVD score was 2.1 in CAV and 0.5 in non-CAV patients (P=.003). Similar degrees of fibrosis and MVD were present even before any evidence of CAV (1yrCAV fibrosis grade 2.5, pre-CAV fibrosis grade 2; 1yrCAV vs CAV P=.75, pre-CAV vs CAV P=.63; 1yrCAV MVD score 2, pre-CAV MVD score 2; 1yrCAV vs CAV P=1, pre-CAV vs CAV P=.91). The degree of MVD correlated with fibrosis (r=0.63, P<.0001) for all EMBs. CONCLUSION: Simultaneous myocardial fibrosis and MVD are noted in CGF secondary to M-S CAV, changes that occur before angiographic CAV. EMBs can reveal significant changes in patients with subsequent development of CAV and may be used to modify the follow-up and treatment for these high-risk patients.

Review of heart transplantation from hepatitis C-positive donors.

Significant scarcity of a donor pool exists for heart transplantation (HT) as the prevalence of patients with end-stage refractory heart failure is increasing exceptionally. With the discovery of effective direct-acting antiviral and favorable short-term outcomes following HT, the hearts from hepatitis C virus (HCV) patient are being utilized to increase the donor pool. Short-term outcomes with regards to graft function, coronary artery vasculopathy, and kidney and liver disease is comparable in HCV-negative recipients undergoing HT from HCV-positive donors compared to HCV-negative donors. A significant high incidence of donor-derived HCV transmission was observed with great success of achieving sustained viral response with the use of direct-acting antivirals. By accepting HCV-positive organs, the donor pool has expanded with younger donors, a shorter waitlist time, and a reduction in waitlist mortality. However, the long-term outcomes and impact of specific HCV genotypes remains to be seen. We reviewed the current literature on HT from HCV-positive donors.

Acute myocardial infarction in heart transplant recipients: An 18-year national study.

Among 11,622,528 acute myocardial infarction (AMI) hospitalizations, 892 had a history of heart transplantation (HT). In comparison to AMI admissions without HT, those with prior HT were more frequently complicated with cardiac arrest (8.3 % vs 5.0 %, p < 0.001), acute non-cardiac organ failure (17.4 % vs 9.4 %) (p < 0.001), lower rates of coronary angiography (55.4 % vs 63.6 %, p < 0.001), comparable rates of percutaneous coronary intervention (38.8 % vs 41.5 %, p = 0.10), higher rates of pulmonary artery catheterization (2.7 % vs 1.1 %, p < 0.001), invasive mechanical ventilation and acute hemodialysis compared to AMI admissions without HT. Compared to AMI admissions without HT, prior HT recipients had higher in-hospital mortality (11.8 % vs 6.2 %, adjusted odds ratio 2.87 [95 % CI 2.23-3.70]; p < 0.001).

Effect of Statin Intensity on the Progression of Cardiac Allograft Vasculopathy.

Background: In the non-transplant population, hyperlipidaemia has shifted from targeting LDL goals to statin intensity-based treatment. It is unknown whether this strategy is also beneficial in cardiac transplantation. Methods: This single-centre retrospective study evaluated the effect of statin use and intensity on time to cardiac allograft vasculopathy (CAV) after cardiac transplantation. Kaplan-Meier and Cox proportional hazards regression survival methods were used to assess the association of statin intensity and median post-transplant LDL on CAV-free survival. Results: The study involved 143 adults (71% men, average follow-up of 25 ± 14 months) who underwent transplant between 2013 and 2017. Mean CAV-free survival was 47.5 months (95% CI [43.1-51.8]), with 29 patients having CAV grade 1 or greater. Median LDL was not associated with time to CAV (p=0.790). CAV-free survival did not differ between intensity groups (p=0.435). Conclusion: Given the non-statistically significant difference in time to CAV with higher intensity statins, the data suggest that advancing moderate- or high-intensity statin after cardiac transplantation may not provide additional long-term clinical benefit. Trial registration: Not applicable.

Imaging Coronary Allograft Vasculopathy with Cardiac PET and Cardiac MRI.

PURPOSE OF REVIEW: Coronary allograft vasculopathy (CAV) is a leading cause of morbidity and mortality in heart transplant patients. It presents a diagnostic challenge as early CAV is often clinically silent, and it affects both epicardial coronary arteries and microvasculature. This review outlines the role of cardiac positron emission tomography (PET) and cardiac magnetic resonance imaging (CMR) in the diagnosis and prognosis of CAV. RECENT FINDINGS: Relative myocardial perfusion imaging (MPI) and quantitative myocardial blood flow using cardiac PET are useful in the diagnosis and prognosis of CAV. Late gadolinium enhancement (LGE) and quantitative measures including myocardial perfusion reserve and mean diastolic rate using CMR are useful in the diagnosis and prognosis of CAV. Cardiac PET is now established as a non-invasive imaging modality for screening and monitoring for CAV, and CMR has demonstrated promise in this application. Further investigation of these modalities is needed with larger, multicenter studies that follow patients serially to demonstrate the clinical implications of using these modalities to detect early CAV and alter therapies to improve patient outcomes.

Detection of cardiac allograft vasculopathy by multi-layer left ventricular longitudinal strain in heart transplant recipients.

Cardiac allograft vasculopathy (CAV) is an obliterative and diffuse type of coronaropathy that develops in the transplanted human heart, representing a major cause of graft failure and mortality. Nowadays the gold standard for the diagnosis of CAV is coronary angiography (CA). Non-invasive CAV detection, especially in the early stages of the disease, is still challenging. Our study aimed to investigate the role of speckle tracking echocardiography (STE), in particular three-layer STE, in predicting CAV at early stages, and if other traditional echocardiographic, clinical or biochemical parameters could relate to CAV. The study population was composed of a total of 33 heart transplanted patients, divided accordingly to the presence or absence of CAV (12 CAV+ , 22 CAV-). All subjects underwent a complete transthoracic echocardiographic examination on the same day of the CA, and all conventional parameters of myocardial function were obtained, including strain values assessed by STE. Strain values were significantly reduced in presence of CAV, at each myocardial layer but in particular the endocardial-epicardial gradient (- 4.15 ± 1.6 vs - 1.7 ± 0.4% < .0001) that was also highly predictive of CAV (AUC at ROC curve 0.97). Among diastolic parameters, the E wave deceleration time (DT) and the mean E/e' ratio were strongly positively associated with CAV. In our population, left ventricular global longitudinal strain (GLS), layer-specific GLS and the endocardial-epicardial LS gradient, E wave DT and E/e' ratio were the best independent non-invasive predictors of CAV.

Percutaneous coronary intervention for coronary allograft vasculopathy with drug-eluting stent in Indian subcontinent: Issues in diagnosis and management.

Coronary allograft vasculopathy fails to give a warning anginal pain due to denervation and often presents with acute coronary syndrome, ventricular dysfunction, or sudden cardiac death. Early diagnosis in a pediatric patient is difficult as it involves invasive coronary angiography or advanced imaging such as intravascular ultrasound or optical coherence tomography. A 12-year-old boy developed acute coronary syndrome, elevated troponins, and right bundle branch block, 5 years after cardiac transplantation and was treated with culprit-vessel angioplasty with a drug-eluting stent. Advanced imaging showed the involvement of nonculprit vessels too. In a detailed literature search, we failed to identify a similar clinical presentation and management in the subcontinent, hence our interest in publishing this report for educational value. Issues in diagnosis, management, prognosis, and prevention are discussed.

Clinical characteristics and in hospital outcomes of heart transplant recipients undergoing percutaneous coronary intervention: Insights from the National Inpatient Sample.

OBJECTIVES: Cardiac transplant patients are at increased risk of Coronary Allograft Vasculopathy which requires percutaneous coronary intervention (PCI). BACKGROUND: We aim to determine national epidemiology describing trends, mortality, and morbidity risks in patients with heart transplant undergoing PCI. METHODS: We used Nationwide Inpatient Sample (NIS) data from 2002 to 2014 to identify adult hospitalizations with PCI using ICD 9 codes. Acute myocardial infarction (AMI), cardiac transplant status and complications were identified using validated ICD-9-CM diagnosis codes. Endpoints were in-hospital mortality and peri-procedural complications. Propensity match analysis was performed to compare the end-points between DES and BMS. RESULTS: Total 8,613,900 patients underwent PCI, of which 1,531(0.002%) patients had prior heart transplant status. Among these 1,531 PCIs, 311(20%) were due to AMI including 125(8%) due to STEMI. 74% of PCIs were done in males and 78% of the PCIs were performed in the 60-79 age group. Out of 1,380 stents placed, 1,090 were DES (79%) and 290 (21%) were BMS. Mortality was higher in the BMS versus DES (8.34% vs. 3.45%, p = .012), CONCLUSION: We concluded that majority of the population who underwent PCI were older males. DES was used more than BMS. The use of BMS is associated with increased mortality, cardiac complications and Acute Kidney Injury requiring dialysis compared with DES which likely is representative of preferential use of DES in these patient population.

Surveillance for cardiac allograft vasculopathy: Practice variations among 50 pediatric heart transplant centers.

BACKGROUND: Coronary allograft vasculopathy (CAV) is a leading cause of mortality after heart transplantation (HT) in children. Variation in CAV screening practices may impact detection rates and patient outcomes. METHODS: Among 50 Pediatric Heart Transplant Society (PHTS) sites from 2001 to 2016, coronary evaluations were classified as angiography or non-invasive testing, and angiograms were designated as routine or symptom based. CAV detection rates stratified by routine vs symptom-based angiograms were calculated. Freedom from CAV and mortality after CAV diagnosis, stratified by study indication, were calculated. RESULTS: A total of 3,442 children had 13,768 coronary evaluations; of these, 97% (n = 13,012) were for routine surveillance, and only 3% (n = 333) were for cause. Over the study period, CAV was detected in 472 patients (14%). Whereas 58% (n = 29) of PHTS sites evaluate by angiography alone, 42% reported supplementing with a non-invasive test, although only 423 non-invasive studies were reported. Angiographic detection of CAV was higher for symptom-based testing than for routine testing (29% vs 4%, p < 0.0001), although routine testing identified a majority of cases (88%; n = 414). The 10-year freedom from CAV was 77% overall. Once CAV is detected, 5-year graft survival was 58%, with lower survival for patients diagnosed after symptoms angiogram than after routine angiogram (30% vs 62%; p < 0.0001). CONCLUSIONS: Development of a robust model for CAV risk should allow low-risk patients to undergo less frequent invasive angiography without adverse impact on CAV detection rates or outcomes.

Impact of combined heart and lung transplantation on bronchiolitis obliterans syndrome, cardiac allograft vasculopathy, and long-term survival.

BACKGROUND: Evidence from animal studies and small case series suggests that primary graft dysfunction occurs less often following combined organ transplantation than following isolated organ transplantation. In this large-scale national registry study, we aimed to investigate whether survival and the rates of bronchiolitis obliterans syndrome (BOS) and coronary allograft vasculopathy (CAV) are affected by simultaneous heart and/or lung transplantation (HLTx). METHODS: Clinical data from the United Network of Organ Sharing database were retrospectively reviewed to identify transplant-naive patients who had undergone heart and/or lung transplantation between 1987 and 2016. The comparisons were conducted for isolated vs combined organ transplant. The outcomes included all-cause mortality, as well as the incidence of BOS and CAV RESULTS: Of the 98,310 patients reviewed, 63,976, 1,189, and 33,145 had received isolated heart transplantation (iHTx) (65%), HLTx (1%), and isolated lung transplantation (iLTx) (34%), respectively. In the early post-operative period, the mortality rates were higher after HLTx than after iHTx or iLTx (on crude and propensity score-matched analyses). However, the adjusted hazard risk for mortality associated with HLTx was significantly lower relative to the iLTx-associated risk beyond 3 years postoperatively, and similar relative to the iHTx-associated risk beyond 7 years postoperatively. On both crude and adjusted analyses, the incidence of BOS and CAV was significantly lower after HLTx than after iHTx or iLTx (p < 0.001 for all comparisons). CONCLUSIONS: Combined (rather than single) organ transplantation may provide immunoprotective benefits enhancing long-term survival and attenuating the risk of BOS and CAV.

PCSK9 inhibition in patients with heart transplantation: A case series.

PCSK9 inhibitors are potent low-density lipoprotein cholesterol-lowering medications. There is a lack of data regarding safety and efficacy of PCSK9 inhibitors in cardiac transplant patients. In this case series, we provide data supporting the low-density lipoprotein-lowering efficacy and short-term safety of PCSK9 inhibitors in three cardiac transplant patients.

Usefulness of dual imaging stress echocardiography for the diagnosis of coronary allograft vasculopathy in heart transplant recipients.

BACKGROUND: Coronary allograft vasculopathy (CAV) is the main factor limiting long-term survival after cardiac transplantation. Dual imaging stress echocardiography with wall motion and Doppler-derived coronary flow reserve (CRF) of the left anterior descending artery (LAD) is a state-of-the-art methodology during dipyridamole stress echocardiography (DiSE). This study involving 74 heart transplanted patients has the purpose to assess the diagnostic value of dipyridamole stress echocardiography with evaluation of wall motion (WM) and Doppler-derived coronary flow reserve for the diagnosis of coronary allograft vasculopathy. METHODS AND RESULTS: All patients underwent DiSE and coronary angiography. Moderate-severe CAV was defined according to International Society of Heart and Lung Transplant (ISHLT) recommended nomenclature for CAV, and CFR < 2 was considered to be impaired. Moderate-severe CAV was present in 11 patients. WM analysis revealed four patients (5%) with rest WM abnormalities. CFR analysis revealed that 40 (54%) individuals had an abnormal result. The combined evaluation of WM analysis and CFR resulted in a sensitivity of 72.7% (95% CI: 39.3 to 92.6%), a specificity of 49.2% (95% CI: 36.5 to 61.9%), a positive predictive value of 20% (95% CI: 9.6 to 36.1%), and negative predictive value of 91.1% (95% CI: 75.1 to 97.6%) for the diagnosis of CAV. CONCLUSIONS: Our results support the inclusion of DiSE performance in Heart transplant follow up protocol. The addition of CFR evaluation offers valuable information to the angiography findings in the detection of CAV and could be helpful in selected patients to adjust the time and indications of coronary angiography.

Right atrial function in pediatric heart transplant patients by echocardiographic strain measurements.

BACKGROUND: CAV is a major cause of mortality in PHTx patients. Research on echocardiographic indices to detect CAV focuses primarily on ventricular function and less is known about RAF. Thus, we primarily sought to evaluate RAF in PHTx patients with CAV. For secondary analysis, we compared RAF between PHTx patients and control patients and evaluated RAF with respect to rejection and surgical type. METHODS: We retrospectively evaluated echocardiography derived RA strain indices in recipients <18 years old and >1 year from time of transplant. The RA strain phases included, reservoir (εs), conduit (εe), pump (εa), and respective strain rate indices (SRs, SRe, SRa). RESULTS: There were 36 PHTx patients and 14 age-, sex-matched control patients. There was a significant reduction in εs, εe, SRs, and SRe (P < 0.001) in the PHTx patients when compared to controls. There was no difference between the CAV (+) and CAV (-) patients with respect to RAF indices. Furthermore, εs, εe, and SRe (P < 0.05) were lower in patients with acute rejection (n = 7) compared to those without (n = 26). Patients with a bi-atrial anastomosis (n = 14) had decreased εs, εa, SRs, SRa (P < 0.05), compared to bi-caval anastomosis (n = 24). CONCLUSION: PHTx patients have decreased RAF compared to healthy children. RAF does not differentiate PHTx patients based on the presence of CAV. RAF is also decreased in PHTx patients with rejection and in those transplanted with a bi-atrial anastomosis.