Depressed Ventricular Function Research Articles

Assessment of B-Natriuretic Peptide Levels After Stage 1 Palliation in Hypoplastic Left Heart Syndrome Patients.

We describe the perioperative BNP trends in patients with HLHS from S1P until interstage discharge to home, death, or S2P. This prospective cohort study includes all newborns with hypoplastic left heart syndrome (HLHS) who underwent Norwood procedures (S1P) at Texas Children's Hospital from April 2018 through April 2019. Our study included 19 newborns with HLHS. There was no interstage mortality; 47% were discharged home prior to the S2P procedure. Nine patients (50%) had higher BNP levels immediately after arrival to the cardiac intensive care unit (ICU) after S1P compared to preoperative levels. BNP levels were higher in those with a shorter duration of mechanical intubation (P = 0.02) and those with moderately depressed right ventricular systolic function in the immediate postoperative period (P = 0.02). RVPAs patients had higher BNP levels (median 3357pg/mL) than mBTTs (median 2862pg/mL), that was not statistically significant (P = 0.4). Despite higher BNP levels in RVPAs in the early postoperative period, these subjects had shorter mechanical ventilation, ICU, and hospital length of stay duration. BNP trends for HLHS patients vary in the postoperative period after S1P. RVPAs had higher BNP levels than mBTTs in the early postoperative period after S1P; however, this was not associated with worse outcomes.

Point of care ultrasonography in the evaluation of myocardial dysfunction due to sepsis.

Background: Septic shock is commonly diagnosed in critically ill patients and is an important cause of mortality. Techniques used to assess fluid responsiveness and hemodynamic profile with physical examination and central venous pressure have been shown to be insufficient. Thus, the importance of other methods, such as bedside ultrasound (POCUS), is evident. The aim of this study was to analyze patients with septic shock who developed left ventricular dysfunction by POCUS. Methods: Prospective study involving 14 patients diagnosed with septic shock, over 18 years old, without previous cardiac pathologies. Clinical, laboratory and imaging data were collected. POCUS was applied by a cardiology resident; the results were compared with those found by an echocardiographer. Results: Variables were compared between patients with normal and depressed ventricular function (VF). Mean arterial pressure was significantly lower in patients with depressed VF (p = 0.01). Vasopressor drug dose and Pro-BNP value were significantly higher in patients with depressed VF (p = 0.01). Regarding the POCUS inter-rater comparison, the variables of left ventricular global systolic function, vena cava index and presence of B line were significantly concordant (p= 0.02; 0.003; 0.002). Conclusions: Patients with depressed VF had a greater severity of shock, suggesting refractoriness, with cardiac dysfunction as a possible aggravating factor, which was visualized only by POCUS and corroborated by higher Pro-BNP values. A short POCUS training is enough for the non-specialist physician to be able to use this resource in the management of these patients.

Successful Pregnancy in A Patient with Fontan Surgery: A Case Report

The Fontan operation is a palliative procedure for patients with several forms of congenital heart disease unsuitable for biventricular circulation, especially tricuspid valve atresia. In this procedure, the vena cava flow is directed to the pulmonary arteries bypassing the ventricle. Pregnant patients with Fontan surgery in their post-surgical history have more maternal risks. We should counsel patients with saturations <85%, depressed ventricular function, refractory arrhythmia, or protein-losing enteropathy against pregnancy (mWHO; IV). In this study, we review a pregnant case that has successful pregnancy and Fontan palliation and lv systolic heart failure (LVEF: 35%) in her past medical history.

Abstract 11687: Diagnosis and Management of High-Risk Pulmonary Embolism in a Term Neonate

Case Presentation: A 5 day old, term infant born to a 39-year-old G1P0 with a history of diet-controlled gestational diabetes mellitus presented with hypernatremic dehydration in the setting of poor feeding. On hospital day 2, the infant suffered a bradycardic cardiac arrest requiring 2 minutes of CPR prior to return of spontaneous circulation. An echocardiogram (Figure 1A) revealed moderately depressed right ventricular (RV) function, elevated RV pressure and a large mobile mass extending from the main pulmonary artery (PA) into the left PA with obstruction of flow. D-dimer was markedly elevated at 2740 ng/mL. A subsequent CT angiogram revealed acute pulmonary emboli in the distal left and right branch PAs (Figure 1B). Additional imaging was obtained and showed dural venous sinus, left portal vein and right cephalic vein thromboses. The infant was managed with a heparin infusion and supportive therapy for the RV dysfunction and pulmonary hypertension including mechanical ventilation, inhaled nitric oxide as well as epinephrine and milrinone infusions. Over the course of two weeks, the infant improved clinically with normalization of RV function. Discussion: Neonatal pulmonary arterial thrombosis is an exceedingly rare phenomenon. The majority of cases described in the literature are associated with either an inherited thrombophilia or with underlying congenital heart disease, which were not found to be present in this case. However, this patient presented with severe dehydration and a history of maternal diabetes, both of which are risk factors for neonatal thrombosis. Fortunately, this patient responded to treatment with anticoagulation alone and did not require more invasive therapies, such as surgical thrombectomy or systemic and catheter-directed thrombolysis. Although rare, neonatal pulmonary arterial thrombosis can occur and delays in recognition and treatment may lead to significant RV dysfunction, morbidity, and even death.

Modeling propofol-induced cardiotoxicity in the isolated-perfused newborn mouse heart.

Infants and children are vulnerable to developing propofol infusion syndrome (PRIS) and young age is a risk factor. Cardiac involvement is often prominent and associated with death. However, the mechanisms of pediatric PRIS are poorly understood because of the paucity of investigation and lack of a gold standard animal model. Unfortunately, in vivo modeling of PRIS in a newborn mouse is not feasible and would be complicated by confounders. Thus, we focused on propofol‐induced cardiotoxicity and aimed to develop an ex‐vivo model in the isolated‐perfused newborn mouse heart. We hypothesized that the model would recapitulate the key cardiac features of PRIS seen in infants and children and would corroborate prior in vitro observations. Isolated perfused newborn mouse hearts were exposed to a toxic dose of propofol or intralipid for 30‐min. Surface electrocardiogram, ventricular contractile force, and oxygen extraction were measured over time. Real‐time multiphoton laser imaging was utilized to quantify calcein and tetramethylrhodamine ethyl ester fluorescence. Propidium iodide uptake was assessed following drug exposure. A toxic dose of propofol rapidly induced dysrhythmias, depressed ventricular contractile function, impaired the mitochondrial membrane potential, and increased open probability of the permeability transition pore in propofol‐exposed hearts without causing cell death. These features mimicked the hallmarks of pediatric PRIS and corroborated prior observations made in isolated newborn cardiomyocyte mitochondria. Thus, acute propofol‐induced cardiotoxicity in the isolated‐perfused developing mouse heart may serve as a relevant ex‐vivo model for pediatric PRIS.

Right Heart Failure Following Left Ventricular Device Implantation: Natural History, Risk Factors, and Outcomes: An Analysis of the STS INTERMACS Database.

Our current understanding of right heart failure (RHF) post-left ventricular assist device (LVAD) is lacking. Recently, a new Interagency Registry for Mechanically Assisted Circulatory Support definition of RHF was introduced. Based on this definition, we investigated natural history, risk factors, and outcomes of post-LVAD RHF. Patients implanted with continuous flow LVAD between June 2, 2014, and June 30, 2016 and registered in the Interagency Registry for Mechanically Assisted Circulatory Support/Society of Thoracic Surgeons Database were included. RHF incidence and predictors, and survival after RHF were assessed. The manifestations of RHF which were separately analyzed were elevated central venous pressure, peripheral edema, ascites, and use of inotropes. Among 5537 LVAD recipients (mean 57±13 years, 49% destination therapy, support 18.9 months) prevalence of 1-month RHF was 24%. Of these, RHF persisted at 12 months in 5.3%. In contrast, de novo RHF, first identified at 3 months, occurred in 5.1% and persisted at 12 months in 17% of these, and at 6 months occurred in 4.8% and persisted at 12 months in 25%. Higher preimplant blood urea nitrogen (ORs,1.03-1.09 per 5 mg/dL increase; P<0.0001), previous tricuspid valve repair/replacement (ORs, 2.01-10.09; P<0.001), severely depressed right ventricular systolic function (ORs,1.17-2.20; P=0.004); and centrifugal versus axial LVAD (ORs,1.15-1.78; P=0.001) represented risk factors for RHC incidence at 3 months. Patients with persistent RHF at 3 months had the lowest 2-year survival (57%) while patients with de novo RHF or RHF which resolved by 3 months had more favorable survival outcomes (75% and 78% at 2 years, respectively; P<0.001). RHF at 1 or 3 months post-LVAD was a common and frequently transient condition, which, if resolved, was associated with relatively favorable prognosis. Conversely, de novo, late RHF post-LVAD (>6 months) was more frequently a persistent disorder and associated with increased mortality. The 1-, 3-, and 6-month time points may be used for RHF assessment and risk stratification in LVAD recipients.

One Heart, Two Problems: Cardiomyopathy and CPVT Secondary to RYR2 Mutation

<h3>Introduction</h3> RYR2 mutations are known to cause catecholaminergic polymorphic ventricular tachycardia (CPVT), an inherited disorder with risk for life threatening atrial (AT) and ventricular (VT) arrhythmias. Individuals with RYR2 mutation less commonly exhibit arrhythmogenic right ventricular cardiomyopathy (ARVC). We report the first case of a pediatric patient with RYR2 mutation, manifesting with CPVT, ARVC, and severe dysfunction requiring heart transplant (HTx). <h3>Case Report</h3> A 4.5 year old male with developmental delay presented after multiple syncopal episodes both at rest and with activity. A Holter showed AT, and he was started on a beta adrenergic antagonist with decreased frequency of syncopal episodes. He then developed sinus bradycardia with marked sinus pauses, prompting dual chamber pacemaker placement at age 6 years. He later exhibited bidirectional VT and whole exome sequencing identified a de novo, likely pathogenic variant in RYR2. He was medically managed until 16 years of age when he had syncope with exercise and was found to have both frequent AT and moderately depressed ventricular function with an ejection fraction of 45%. Given recurrent syncope with exercise, he underwent uncomplicated sympathectomy and optimization of his medical antiarrhythmic management. Over the next few months, he developed progressive heart failure symptoms and was admitted with worsening biventricular dysfunction and tachyarrhythmias. During his admission, he had a cardiac arrest with initial monitoring demonstrating VT, and was placed on extracorporeal membrane oxygenation support. He was transitioned to a durable left ventricular assist device (VAD), and was eventually listed for HTx. His VAD course was complicated by RV dysfunction needing PDE5 inhibitor; his arrhythmia burden was managed on flecainide, sotalol, and nadolol. After 80 days of VAD support, he underwent HTx. Pathology of his explanted heart revealed biventricular fibrofatty myocardial replacement consistent with ARVC with prominent LV involvement. <h3>Summary</h3> This case highlights an expanded spectrum of RYR2 mutation related disease which may include prominent atrial arrhythmias, ARVC, and developmental delay. Patients with CPVT and RYR2 mutation warrant close monitoring for ventricular dysfunction, especially if there is a significant arrhythmia burden.

Right-sided heart reperfusion ("Berlin bridge technique") for right ventricle support during left ventricular assist device (LVAD) implantation.

Right-sided heart reperfusion ("Berlin bridge technique") for right ventricle support during left ventricular assist device (LVAD) implantation.

Association of Myocardial Muscle Non-Compaction and Multiple Ventricular Septal Defects by Echocardiography.

The aim of this study is to examine the possible high association between multiple ventricular septal defect (mVSDs) and noncompaction cardiomyopathy (NCM) as same embryological origin, and the effect of depressed ventricular function in NCM cases during the follow-up, using echocardiography. A total of 150 patients with mVSDs were diagnosed in a single center in Saudi Arabia; 40 cases with isolated or associated with minor congenital heart disease were recruited. Three specialist echocardiography consultants confirmed the NCM diagnosis separately using Jenni, Chin and Patrick criteria, and myocardial function was estimated by ejection fraction at admission and at follow-up after surgery. Stata-14 to analyze the data was used. In our cohort of 40 cases with mVSD (median age at diagnosis = 0.5 years; mean follow-up = 4.84 years), 13(33%) had criteria of non-compaction confirmed by the three specialist consultants. All were operated by surgery and 11 hybrid approach (interventional & surgery). A significant relationship between abnormal trabeculations and mVSD with or without non-compaction was observed, 34% vs 66% respectively (p < 0.03, Fisher's exact test). A repeated-measures t-test found the difference between follow-up and preoperative ejection-fractions to be statistically significant (t (39) = 2.07, p < 0.04). Further, the myocardial function in the mVSD non-compaction group normalized substantially postoperatively compared with preoperative assessment (mean difference (MD) 11.77, 95% CI: 4.40-19.14), whilst the mVSD group with normal myocardium had no significant change in the myocardium function (MD 0.74, 95% CI: -4.10-5.58). Thus, treatment outcome appears better in the mVSD non-compaction group than their peers with normal myocardium. Acknowledging the lack of genetic data, it is evident the high incidence of non-compaction in this cohort of patients with mVSD and supports our hypothesis of embryonic/genetic link, unlikely to be explained by acquired cardiomyopathy.

Posttraumatic pneumonectomy and management of severely contaminated pleural space.

Posttraumatic pneumonectomy and management of severely contaminated pleural space.

31 Cardiac magnetic resonance findings in patients with Type 1 myotonic dystrophy

Abstract Aims Heart disease is a major determinant of prognosis in type 1 myotonic dystrophy (DM1), second only to respiratory complications. Cardiac imaging, possibly including cardiac magnetic resonance (CMR), is recommended in patients with DM1. However, limited information is available on CMR findings and their prognostic significance in DM1. Methods and results We identified all patients with DM1 evaluated from 2009 to 2020 in a CMR laboratory with an established collaboration with a Neuromuscular Disorder Unit. Thirty-four patients were retrieved (21 males, aged 45 ± 12). By the time of CMR examination, 90% had neuromuscular symptoms (mean duration 17 ± 13 years), 13 (38%) had previous reports of atrioventricular block (n = 12 1st degree, n = 1 2nd degree type 1), 30 (88%) of intraventricular conduction disturbances (n = 5 left bundle branch block, n = 5 right bundle branch block, n = 3 left anterior fascicular block, n = 17 other non-specific or incomplete intraventricular conduction delay), 4 (12%) of atrial fibrillation or flutter. No patient had a device. At CMR, 5 (15%) patients had left ventricular (LV) systolic dysfunction [LV ejection fraction (LVEF) &amp;lt;50%] and 4 (12%) a depressed right ventricular (RV) function (RVEF &amp;lt;50%). Compared to age- and sex-specific reference values for our laboratory (Figure 1 left), 12 (35%) patients showed a decreased LV end-diastolic volume index (LVEDVi), 7 (21%) a decreased LV mass index (LVMi), and 29 (85%) a decreased LVMi/LVEDVi ratio. Nine (26%) patients had mid-wall late gadolinium enhancement (LGE, mean extent 4.5 ± 2.0% of LVM; n = 8 septal, n = 4 inferolateral, n = 2 inferior, n = 1 anterolateral, see Figure 1 middle), and 14 (41%) some areas of fatty infiltration (n = 9 involving the LV, n = 13 the RV). Native T1 in the interventricular septum (1,041 ± 53 ms) approached the upper reference limit (1089 ms), and the extracellular volume was slightly increased (33 ± 2%, reference values &amp;lt;30%). Over a median follow-up of 2.5 years (interquartile interval: 1.5–4.0), 2 (6%) patients died for infectious and respiratory complications, 5 (15%) underwent device implantation (n = 4 PM; n = 1 ICD), and 4 (12%) had a documentation of high-risk (Lown class ≥4) ventricular ectopic beats (VEBs). Among all CMR variables collected, higher values of LVMi/LVEDVi ratio emerged as univariate predictor of all-cause death (P = 0.044). At logistic regression analysis, anteroseptal wall thickness was associated with the need for device implantation (P = 0.028), while LGE mass was associated with high-risk VEBs (P = 0.026) (Figure 1 right). Conclusions Patients with DM1 display several structural and functional cardiac abnormalities, with variable degrees of cardiac muscle hypotrophy, fibrosis, and fatty infiltration. The possibility to predict the need for device implantation, ventricular arrhythmias, and all-cause or cardiovascular mortality should be verified in larger cohorts.

Abstract 12599: Pushing the Boundaries of Heart Transplantation in a Patient With Giant Cell Myocarditis and Pulmonary Embolism

A 47-year-old woman with a remote history of hormone receptor positive breast cancer presented to our hospital with dyspnea and malaise. She was hypotensive and had an S3 gallop with cool extremities. Electrocardiogram demonstrated atrioventricular dissociation with a wide underlying ventricular escape. Transthoracic echocardiography revealed a left ventricular ejection fraction of 15% and severely depressed right ventricular function with normal biventricular dimensions.She emergently went for temporary transvenous pacemaker placement and a right heart catheterization with endomyocardial biopsy. This revealed elevated biventricular filling pressures (central venous pressure = 16 mmHg; pulmonary capillary wedge pressure = 19 mmHg) and a low cardiac index (2.0 L/min/kg 2 ).Her hemodynamics deteriorated despite inotropes and an intra-aortic balloon pump, prompting escalation to veno-arterial extracorporeal membrane oxygenation. She was initiated on pulse dose steroids for 3 days, followed by a slow taper. Over the course of 24 hours, she had worsening right ventricular function and developed episodic ventricular tachyarrhythmias requiring recurrent defibrillation. She was escalated to CentriMag biventricular support. The endomyocardial biopsy revealed severe giant cell myocarditis with &gt;25% destruction of the sampled myocardium. After an expedited transplant committee review, the patient was listed for heart transplantation (HT).Three days after HT listing, she developed acute hypoxemia and profound hypotension. CT angiography of the chest identified multiple pulmonary emboli (PE), including a centrally located thrombus causing partial obstruction of the right pulmonary artery. Despite this complication, the patient proceeded to a combined pulmonary embolectomy and HT. She was discharged home from the hospital post HT and at 10-week follow-up demonstrated excellent functional rehabilitation.Although PE is a relative contraindication for heart transplantation, the reversible nature of her pathology and the benefit of avoiding time-dependent pulmonary vascular remodeling favored early transplantation. PE in HT recipients is far less well characterized and has important implications for management and prognosis.

A biomarker for amniotic fluid embolism: the search continues.

Research into the pregnancy associated cytokine storm-like condition historically known as amniotic fluid embolism (AFE) has been hampered by a lack of unique diagnostic criteria. In its classic form, the clinical presentation of this condition is unmistakable. In less classic presentations, each of the clinical hallmarks of AFE (depressed ventricular function, lung injury and coagulopathy) may, in isolation, be seen in other obstetric conditions. Indeed, much of the confusion arising from previously published case series purporting to describe women with AFE appears to be the result of the inclusion of patients with other conditions. (Clark SL et al Obstet Gynecol 2014:123: 337-48) Identification of a reliable, objective biomarker specific to AFE is badly needed.

Cardiac magnetic resonance findings in patients with type 1 myotonic dystrophy

Abstract Funding Acknowledgements Type of funding sources: None. Background Heart disease is a major determinant of prognosis in type 1 myotonic dystrophy (DM1), second only to respiratory complications. Cardiac imaging, possibly including cardiac magnetic resonance (CMR), is recommended in patients with DM1. However, limited information is available on CMR findings and their prognostic significance in DM1. Methods We identified all patients with DM1 evaluated from 2009 to 2020 in a CMR laboratory with an established collaboration with a Neuromuscular Disorder Unit. Results Thirty-four patients were retrieved (21 males, aged 45 ± 12). At the time of CMR examination, 97% had neuromuscular symptoms (mean duration 16 ± 13 years), 12 (35%) presented with atrioventricular block (n = 11 1st degree, n = 1 2nd degree type 1), 15 (44%) with intraventricular conduction disturbances (n = 5 left bundle branch block, n = 5 right bundle branch block, n = 3 left anterior fascicular block, n = 2 other non-specific intraventricular conduction delay), 4 (12%) with atrial fibrillation or flutter. No patient had a device. At CMR, 5 (15%) patients had left ventricular (LV) systolic dysfunction (LV ejection fraction [LVEF] &amp;lt;50%) and 5 (15%) a depressed right ventricular (RV) function (RVEF &amp;lt;50%). Compared to age- and sex-specific reference values for our laboratory, 12 (35%) patients showed a decreased LV end-diastolic volume index (LVEDVi), 7 (21%) a decreased LV mass index (LVMi), and 29 (83%) a decreased LVMi/LVEDVi ratio. Nine (26%) patients had mid-wall late gadolinium enhancement (LGE, mean extent 4.5 ± 2.0% of LVM; n = 8 septal, n = 4 inferolateral, n = 2 inferior, n = 1 anterolateral), and 14 (40%) some areas of fatty infiltration (n = 9 involving the LV, n = 13 the RV). Native T1 in the interventricular septum (1,041 ± 53 ms) approached the upper reference limit (1,089 ms), and the extracellular volume was slightly increased (33 ± 2%, reference values &amp;lt;30%). Over a median follow-up of 3.3 years (interquartile interval 1.6-4.7), 2 (6%) patients died, one for infectious and respiratory complications and the other for unknown causes, 5 (15%) patients underwent pacemaker implantation for conduction disturbances, and 4 (12%) had a documentation of high-risk (Lown class ≥4) ventricular ectopic beats (VEBs). Among all CMR variables collected, higher values of LVMi/LVEDVi ratio emerged as univariate predictor of all-cause death (p = 0.044). At logistic regression analysis, anteroseptal wall thickness was associated with the need for pacemaker implantation (p = 0.028), while LGE mass was associated with high-risk VEBs (p = 0.026). Conclusions Patients with DM1 display several structural and functional cardiac abnormalities, with variable degrees of cardiac muscle hypotrophy, fibrosis and fatty infiltration. The possibility to predict the need for pacemaker implantation, ventricular arrhythmias and all-cause or cardiovascular mortality should be verified in larger cohorts.

Clinical, Electrocardiographic and Echocardiographic Profile of Ischemic Cardiomyopathy: An analysis of 100 cases.

Background: Ischemic heart disease (IHD) is one of the leading cause of morbidity and mortality worldwide. Ischemic cardiomyopathy (ICM) is a delayed complication of IHD that arises as dilated cardiomyopathy with depressed ventricular function, which cannot be attributed entirely to coronary artery obstruction or ischemic injury.&#x0D; Objectives: To evaluate the clinical, electrocardiographic and echocardiographic profile of patients presenting with ischemic cardiomyopathy. Methods: In this cross sectional observational study 100 patients of ischemic cardiomyopathy admitted in hospital or visited OPD in NICVD, Dhaka from March’15 to Sept’15 were studied. Enrollment of the patients were done after fulfilling the inclusion and exclusion criteria. Clinical, electrocardiographic and echocardiographic data were collected then data analysis was done.&#x0D; Results: Data analysis of 100 patients was showed age range was 40-80 years and mean age was 61.4±7.9 years. 79% subjects were male. Most common symptoms were dyspnea (93%), chest pain(73%), palpitation (39%) and edema (23%). Most patients were in NYHA functional class lV (43%). 64% cases had history of anterior myocardial infarction (MI), 22% had inferior MI, 25% had H/O PTCA and 7% had CABG. 71% subjects had tachycardia, 65% had lungs basal rales, 56% had systolic blood pressure below 100 mmhg and 25% had edema. ECG findings was as follows sinus rhythm (85%), Sinus tachycardia 71%, AF 15%, LBBB 34%, RBBB 12%, pathological Q in anterior surface 65% and inferior surface 21%, non specific ST-T changes 41% and PVCs was found in 17%.On echocardiography ,anterior wall hypokinesia was seen in 52% and global hypokinesia in 43%. Mean left ventricular ejection fraction (LVEF) was 31±5.9% and mean left ventricular internal diastolic diameter (LVIDd) was 6.5±0.4 cm. (59%) subjects had mitral regurgitation (MR) grade-l and 20% had MR grade-ll.&#x0D; Conclusion: The clinical presentation of ischemic cardiomyopathy varies from patient to patient. Severity of symptoms correlates with severity of left ventricular systolic dysfunction, left ventricular diameter and mitral regurgitation grade . Anterior Myocardial infarction has more chance to develop ischemic cardiomyopathy.&#x0D; Bangladesh Heart Journal 2020; 35(2) : 121-127

Assisting the Heart to Assist the Lungs: LVAD Support in Restrictive Physiology and Pulmonary Hypertension

<h3>Introduction</h3> Pulmonary hypertension (PH) is a risk factor for mortality after heart transplantation (HTx). We present a case of treatment of post-capillary PH in a small pediatric patient with restrictive physiology using continuous flow ventricular assist device (VAD) support to achieve candidacy for HTx. <h3>Case Report</h3> A 4.5 year old female (0.57m²) with a history of acute myeloid leukemia and anthracycline chemotherapy in infancy developed mildly depressed left ventricular (LV) function at 2 years of age. Hemodynamic catheterization (cath) showed pre- and post-capillary PH secondary to LV diastolic dysfunction and restrictive physiology (Table). She was cautiously initiated on PDE5 inhibitor therapy (sildenafil) while maintaining LV reverse remodeling therapies. Her heart failure (HF) symptoms and biventricular systolic function worsened over the subsequent 2 years. Subsequent caths showed increasing PVRi, which was responsive to 100% FiO2 and inhaled nitric oxide (iNO), but also accompanied by increase in LV end-diastolic pressures (LVEDP). She was transitioned to endothelin receptor antagonist therapy (bosentan), but continued to worsen clinically, requiring initiation of milrinone. Due to her underlying restrictive physiology leading to worsening PH and severely depressed right ventricular (RV) function, a HeartWare HVAD (cannulation LV to Aorta) was implanted to allow aggressive optimization of PH therapies with simultaneous LV decompression. This enabled up-titration of bosentan as well as re-addition of sildenafil. After PH therapies including VAD implantation, PVRi decreased from a high of 10.7 WU.m2 to 4.7 WU.m2, and marked decline of LVEDP from 22 mmHg pre-VAD to 5 mmHg. She underwent HTx 6 months after VAD implantation, and was discharged home 12 days post-HTx on sildenafil. <h3>Summary</h3> VAD support in tandem with optimal PH therapies is a feasible strategy to treat post-capillary PH, optimize pulmonary vasculature to reduce RV dysfunction, and enable a successful bridge to HTx.

Resolution of Nodular Regenerative Hyperplasia Following Heart Transplant

Introduction Liver abnormalities are common in patients with advanced heart failure. Chronically elevated right sided pressures and resulting hepatic congestion can cause progressive parenchymal changes including nodular regenerative hyperplasia (NRH) and fibrosis. Liver dysfunction and/or liver nodularity on imaging necessitates transjugular liver biopsy (TJLB) to assess for irreversible liver damage. Little is known about post-heart transplant (HT) liver architecture changes. We present a patient with nodular liver parenchyma due to NRH who experienced resolution of NRH after HT. Case Report A 43 year old man with non-ischemic cardiomyopathy, ejection fraction of 15%, and severely depressed right ventricular function was evaluated for HT. Liver function tests (LFT) were abnormal (AST 46, ALT 35, total bilirubin 1.6, alkaline phosphatase (AP) 141) and ultrasound revealed liver nodularity. Right heart catheterization (RHC) revealed elevated pressures (right atrium (RA) 18 mmHg, pulmonary artery (PA) 69/30 mmHg). TJLB had zone 3 sinusoidal dilation and congestion, stage 1 fibrosis, and NRH. 10 months post-HT, LFTs remained elevated (AST 41, ALT 71, total bilirubin 0.8, AP 121) while RHC showed improvement (RA 8 mmHg, PA 35/8 mmHg). Magnetic resonance elastography (MRE) was notable for hepatic stiffness (3 kPa) consistent with stage 1-2 fibrosis. Repeat TJLB showed minimal zone 3 fibrosis (stage 1) without NRH. 32 months post-HT, LFTs were improved (AST 42, ALT 49, total bilirubin 1.1, AP 65), repeat MRE showed slight reduction in stiffness (2.8 kPa), and RHC pressures were stable (RA 11 mmHg, PA 39/21 mmHg). Summary This case shows that congestive hepatopathy induced NRH may resolve after HT. NRH, characterized by focal liver regeneration without evidence of fibrosis, results from dysfunctional hepatic blood flow. Liver regions receiving normal or increased blood flow hypertrophy and form nodules while hypoperfused areas atrophy. Clinically, NRH can lead to portal hypertension and associated complications. TJLB in HT candidates allows closer examination of parenchymal injury. The goal of TJLB is to differentiate reversible liver injury from permanent damage, as the latter is a contraindication to HT. There is a paucity of information about post- HT parenchymal changes and prospective case series are needed to characterize hepatic recovery following HT.

Giant Cell Myocarditis after Orthotopic Heart Transplant

<h3>Introduction</h3> Giant cell myocarditis (GCM) is a rare, T-lymphocyte driven, inflammatory disease that leads to severe myocardial dysfunction and high morbidity. De novo occurrence of GCM after orthotopic heart transplant has not been reported and there is currently little evidence to guide management. Here we present a case of new onset GCM after orthotopic heart transplant (OHT) successfully treated with high dose steroids. <h3>Case Report</h3> A 66-year-old male with end stage heart failure due to ischemic cardiomyopathy underwent OHT complicated by cytomegalovirus viremia treated with valganciclovir. His immunosuppression regimen included tacrolimus 1.5mg twice a day, mycophenolate mofetil 1g twice a day, and prednisone 15mg daily. Three months after transplant, he was found to have acute cellular rejection (grade 2R) on routine endomyocardial biopsy (EMB) and treated with pulse dose steroids. EMB one month later demonstrated a focus of giant cell and histiocytes with surrounding interstitial and perivascular inflammation, suggesting a diagnosis of GCM (Figure 1A). An echocardiogram showed an ejection fraction of 54% without wall motion abnormalities, and mildly depressed right ventricular systolic function. He was treated with methylprednisone 1g daily for three days followed by a prolonged prednisone taper in addition to sirolimus 0.5 mg daily and tacrolimus 1.5 mg twice a day. Giant cell was absent on EMB one month later, suggesting response to therapy (Figure 1B). Patient was doing well without complications six weeks after diagnosis. <h3>Summary</h3> We present a case of GCM diagnosed on EMB in a patient who is status post orthotopic heart transplant, successfully treated with high dose steroids and immunosuppressive therapy. It is important to note that there were no giant cells in his heart explant and that we cannot definitely rule out sarcoidosis. This case however highlights the importance of early diagnosis of GCM and prompt treatment. Further studies are needed to determine the optimal treatment strategy for these patients.

The Use of Cardiac Resynchronization Therapy for Heart Failure in Patients One Year and Under

Introduction Cardiac resynchronization (CRT) therapy with biventricular (biV) or multi-site ventricular pacing has been shown to improve cardiac function and outcomes in adult patients with cardiomyopathy. However, little is known about the benefits of this therapy in infants with complex congenital heart disease. We present 4 inotrope-dependent heart failure patients being considered for heart transplant, 1 year or younger, from our institution who avoided heart transplantation following the initiation of CRT. Case Report Patient 1 has neonatal lupus and dilated cardiomyopathy with severely decreased ejection fraction (EF). Following a cardiac arrest at 2 months, she was unable to wean off ECMO and underwent CRT pacemaker (PM) placement. She weaned off ECMO in 48 hours and inotropes after 15 days. Her EF normalized 7 months after CRT upgrade. QRS improved from 126 to 108 milliseconds (ms) Patient 2 has double inlet left ventricle (DILV) with heart block requiring a dual chamber PM. He then developed dyssynchronous function that prevented him from second stage repair, so he was upgraded to a CRT pacing system at 7 months. 4 days later, his echocardiogram (echo) showed improved synchrony, and he was able to have his surgery at 12 months. QRS improved from 158 to 132 ms. Patient 3 has DILV and congenital heart block who had a single chamber PM placed. Following a surgical procedure, she suffered hemodynamic compromise and remained on ECMO. Her PM was converted to a dual chamber system and her EF normalized in 7 days; however, 5 weeks later she had severely diminished EF. After multiple attempts to wean inotropes, she was upgraded to a CRT system at 17 months. Her EF significantly improved in 10 days, and she was off inotropes 21 days later. QRS improved from 180 to 130 ms. Patient 4 developed heart block after cardiac fibroma resection and had a dual chamber PM placed. Despite this, she remained on inotropes and was upgraded to a biV CRT system at 3 months. Her EF never normalized, but she weaned off inotropes after 64 days. QRS improved from 252 to 148 ms. Unfortunately, she suffered a fatal seizure at 2 years. Summary In this series of infants with severely depressed ventricular function, cardiac resynchronization improved ejection fraction and prevented the need for heart transplantation. Following CRT initiation, all patients were weaned off inotropic support and discharged without heart transplantation.

ECG of the Month: Wide Complex Tachycardia in an Adult Patient with Surgically Corrected Congenital Heart Disease

A 27-year-old man with history of hypoplastic left-sided heart syndrome, status post-Fontan palliation surgery, and depressed right-sided ventricular function presented to the emergency department with acute-onset chest pain and shortness of breath while walking up steps to his home 90 minutes before presentation. He was ill appearing and diaphoretic on arrival. He was alert but had delayed capillary refill and perioral cyanosis. His initial vital signs were significant for pulse rate of 204 beats/min, respiratory rate of 22 breaths/min, blood pressure 132/75 mm Hg, and oxygen saturation of 88% on 3 L of nasal cannula oxygen.