Case Illustration of the Natural History of Left Dominant Arrhythmogenic Cardiomyopathy.

Background: Arrhythmogenic left ventricular cardiomyopathy is an increasingly recognized cause of recurrent myocarditis, a mimicker of acute coronary syndrome, and an important cause of malignant ventricular arrythmias and heart failure. Desmoplakin is a protein that is critical to maintaining the structural integrity of the myocardium. Disruption of desmoplakin leads to fibrofatty infiltration of the myocardium which leads to congestive heart failure, cardiac arrhythmias, and sudden cardiac death. However, desmoplakin cardiomyopathy is often misdiagnosed, resulting in significant morbidity and mortality. We report 2 contrasting cases illustrating the natural history-hot and cold phases-of arrhythmogenic left ventricular cardiomyopathy. Case Series: The first case demonstrates a common phenotypic presentation of desmoplakin cardiomyopathy manifested as recurrent myocarditis and myocardial injury representing the hot phase. The second case is an undulating course of chronic systolic heart failure and ventricular arrhythmias representing the cold phase. Conclusion: Arrhythmogenic cardiomyopathy manifests as a spectrum of disease processes that involve the right, left, or both ventricles. Mutations in the desmoplakin gene are often associated with a left dominant ventricular cardiomyopathy. Diagnosis remains difficult as the condition has no signature clinical presentation, and imaging findings are variable.

Potential Prognostic Value of Native T1 in Pulmonary Hypertension Patients.

Native T1, extracellular volume fraction (ECV), and late gadolinium enhancement (LGE) characterize myocardial tissue and relate to patient prognosis in a variety of diseases, including pulmonary hypertension. The purpose of this study was to evaluate if left ventricle (LV) fibrosis measurements have prognostic value for cardiac outcomes in pulmonary hypertension subgroups. 54 patients with suspected pulmonary hypertension underwent right-heart catheterization and were classified into pulmonary hypertension subgroups: pre-capillary component (PreCompPH) and isolated post-capillary (IpcPH). Cardiac magnetic resonance imaging (MRI) scans were performed with the acquisition of balanced cine steady-state free precession, native T1, and LGE pulse sequences to measure cardiac volumes and myocardial fibrosis. Associations between cardiac events and cardiac MRI measurements were analyzed within PreCompPH and IpcPH patients. IpcPH: LV native T1 was higher in patients who experienced a cardiac event within two years vs. those who did not. In patients with LV native T1 > 1050 ms, the rate of cardiac events was higher. ECV and quantitative LGE did not differ between groups. PreCompPH: native T1, ECV, and quantitative/qualitative LGE did not differ between patients who experienced a cardiac event within two years vs. those who did not. LV native T1 may have potential value for forecasting cardiac events in IpcPH, but not in PreCompPH, patients.

Quantitative Assessment of Regional Pulmonary Transit Times in Pulmonary Hypertension.

BACKGROUND: Pulmonary hypertension (PH) contributes to restricted flow through the pulmonary circulation characterized by elevated mean pulmonary artery pressure acquired from invasive right heart catheterization (RHC). MRI may provide a noninvasive alternative for diagnosis and characterization of PH. PURPOSE: To characterize PH via quantification of regional pulmonary transit times (rPTT). STUDY TYPE: Retrospective. POPULATION: A total of 43 patients (58% female); 24 controls (33% female). RHC-confirmed patients classified as World Health Organization (WHO) subgroups 1-4. FIELD STRENGTH/SEQUENCE: A 1.5 T/time-resolved contrast-enhanced MR Angiography (CE-MRA). ASSESSMENT: CE-MRA data volumes were combined into a 4D matrix (3D resolution + time). Contrast agent arrival time was defined as the peak in the signal-intensity curve generated for each voxel. Average arrival times within a vessel region of interest (ROI) were normalized to the main pulmonary artery ROI (t0 ) for eight regions to define rPTT for all subjects. Subgroup analysis included grouping the four arterial and four venous regions. Intraclass correlation analysis completed for reproducibility. STATISTICAL TESTS: Analysis of covariance with age as covariate. A priori Student's t-tests or Wilcoxon rank-sum test; α = 0.05. Results compared to controls unless noted. Significant without listing P value. ICC ran as two-way absolute agreement model with two observers. RESULTS: PH patients demonstrated elevated rPTT in all vascular regions; average rPTT increase in arterial and venous branches was 0.85 ± 0.15 seconds (47.7%) and 1.0 ± 0.18 seconds (16.9%), respectively. Arterial rPTT was increased for all WHO subgroups; venous regions were elevated for subgroups 2 and 4 (group 1, P = 0.86; group 3, P = 0.32). No significant rPTT differences were found between subgroups (P = 0.094-0.94). Individual vessel ICC values ranged from 0.58 to 0.97. DATA CONCLUSION: Noninvasive assessment of PH using standard-of-care time-resolved CE-MRA can detect increased rPTT in PH patients of varying phenotypes compared to controls. LEVEL OF EVIDENCE: 1 TECHNICAL EFFICACY: Stage 3.

Evaluation of Pulmonary Hypertension Using 4D Flow MRI.

BACKGROUND: Cardiac magnetic resonance imaging (MRI) is becoming an alternative to right heart catheterization (RHC) for evaluating pulmonary hypertension (PH). A need exists to further evaluate cardiac MRI's ability to characterize PH. PURPOSE: To evaluate the potential for four-dimensional (4D) flow MRI-derived pulmonary artery velocities to characterize PH. STUDY TYPE: Prospective case-control. POPULATION: Fifty-four PH patients (56% female); 25 controls (36% female). FIELD STRENGTH/SEQUENCE: 1.5 T; gradient recalled echo 4D flow and balanced steady-state free precession cardiac cine. ASSESSMENT: RHC was used to derive patients' pulmonary vascular resistance (PVR). 4D flow measured blood velocities at the main, left, and right pulmonary arteries (MPA, LPA, and RPA); cine measured ejection fraction, end diastolic, and end systolic volumes (EF, EDV, and ESV). EDV and ESV were normalized (indexed) to body surface area (ESVI and EDVI). Parameters were evaluated between, and within, PH subgroups: pulmonary arterial hypertension (PAH); PH due to left heart disease (PH-LHD)/chronic lung disease (PH-CLD)/or chronic thrombo-emboli (CTE-PH). STATISTICAL TESTS: Analysis of variance and Kruskal-Wallis tests compared parameters between subgroups. Pearson's r assessed velocity, PVR, and volume correlations. Significance definition: P < 0.05. RESULTS: PAH peak and mean velocities were significantly lower than in controls at the LPA (36 ± 12 cm/second and 20 ± 4 cm/second vs. 59 ± 15 cm/second and 32 ± 9 cm/second). At the RPA, mean velocities were significantly lower in PAH vs. controls (27 ± 6 cm/second vs. 40 ± 9 cm/second). Peak velocities significantly correlated with right ventricular EF at the MPA (r = 0.286), RPA (r = 0.400), and LPA (r = 0.401). Peak velocity significantly correlated with right ventricular ESVI at the RPA (r = -0.355) and LPA (r = -0.316). Significant correlations between peak velocities and PVR were moderate at the LPA in PAH (r = -0.641) and in PH-LHD (r = -0.606) patients, and at the MPA in PH-CLD (r = -0.728). CTE-PH showed non-significant correlations between peak velocity and PVR at all locations. DATA CONCLUSION: Preliminary findings suggest 4D flow can identify PAH and track PVR changes. LEVEL OF EVIDENCE: 1 TECHNICAL EFFICACY: Stage 5.

Left Ventricular Fibrosis Assessment by Native T1, ECV, and LGE in Pulmonary Hypertension Patients.

Cardiac magnetic resonance imaging (MRI) is emerging as an alternative to right heart catheterization for the evaluation of pulmonary hypertension (PH) patients. The aim of this study was to compare cardiac MRI-derived left ventricle fibrosis indices between pre-capillary PH (PrePH) and isolated post-capillary PH (IpcPH) patients and assess their associations with measures of ventricle function. Global and segmental late gadolinium enhancement (LGE), longitudinal relaxation time (native T1) maps, and extracellular volume fraction (ECV) were compared among healthy controls (N = 25; 37% female; 52 ± 13 years), PH patients (N = 48; 60% female; 60 ± 14 years), and PH subgroups (PrePH: N = 29; 65% female; 55 ± 12 years, IpcPH: N = 19; 53% female; 66 ± 13 years). Cardiac cine measured ejection fraction, end diastolic, and end systolic volumes and were assessed for correlations with fibrosis. LGE mural location was qualitatively assessed on a segmental basis for all subjects. PrePH patients had elevated (apical-, mid-antero-, and mid-infero) septal left ventricle native T1 values (1080 ± 74 ms, 1077 ± 39 ms, and 1082 ± 47 ms) compared to IpcPH patients (1028 ± 53 ms, 1046 ± 36 ms, 1051 ± 44 ms) (p < 0.05). PrePH had a higher amount of insertional point LGE (69%) and LGE patterns characteristic of non-vascular fibrosis (77%) compared to IpcPH (37% and 46%, respectively) (p < 0.05; p < 0.05). Assessment of global LGE, native T1, and ECV burdens did not show a statistically significant difference between PrePH (1.9 ± 2.7%, 1056.2 ± 36.3 ms, 31.2 ± 3.7%) and IpcPH (2.7 ± 2.7%, 1042.4 ± 28.1 ms, 30.7 ± 4.7%) (p = 0.102; p = 0.229 p = 0.756). Global native T1 and ECV were higher in patients (1050.9 ± 33.8 and 31.0 ± 4.1%) than controls (28.2 ± 3.7% and 1012.9 ± 29.4 ms) (p < 0.05). Cardiac MRI-based tissue characterization may augment understanding of cardiac involvement and become a tool to facilitate PH patient classification.

COVID-19 and the Cardiovascular System.

COVID-19, a symptom complex of respiratory failure induced by a highly infectious pathogen, severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), has been classified as a pandemic. As of April 15, 2020, there have been 2 million people diagnosed with the viral infection and 130 000 deaths globally. It is highly likely that the number of infections is underrepresented secondary to variations in testing and reporting strategies globally. In this short review, we aim to summarize the current understanding of SARS-CoV-2 as it pertains to cardiovascular disease. We discuss the basis of cardiac pathophysiology and address some of the clinical scenarios that cardiovascular physicians may face. We introduce the concept of conservative management of acute coronary syndromes and address some complications such as myocarditis, heart failure, and cardiac arrhythmias that may be relevant for the management of patients presenting with COVID-19.

Hepatic infarction and acute pancreatitis: a case report and review of the literature.

Hepatic infarction is rare due to the unique dual hepatic blood supply from the hepatic artery and the portal vein. Herein, we report a case of hepatic infarction that occurred as a complication of acute pancreatitis. The patient was a 58-year-old male with past medical history of chronic alcoholism, who presented with epigastric abdominal pain, nausea, and vomiting. Hepatic infarction was diagnosed with computed tomography of the abdomen and pelvis without contrast, which revealed suspicion of splenic vein thrombosis and peripancreatic fat stranding along with a wedge-shaped, peripheral hypo density in the right hepatic lobe with typical morphology for hepatic infarction.

Early-onset de novo invasive pulmonary aspergillosis in an orthotopic heart transplant recipient.

Invasive aspergillosis generally occurs during the first 1-6 months after heart transplantation. It has been rarely seen in the first 2 weeks postcardiac transplant. We herein describe a unique case of invasive pulmonary aspergillosis (IPA) diagnosed on day 9 postorthotopic heart transplantation. The known risk factors for IPA in cardiac transplant recipients were not identified in our case. The organ recipients from the same donor did not report Aspergillus infection. Hospital environmental samplings failed to demonstrate Aspergillus spores in the patient's room and his adjacent rooms. A diagnosis of early-onset de novo IPA was made. The patient initially received combined antifungal therapy (voriconazole plus micafungin), followed by voriconazole maintenance monotherapy with favorable clinical outcome.

Adenoviral nephritis in a renal transplant recipient: Case report and literature review.

Adenovirus (AdV) infections in transplant recipients may cause invasive disease. We present a case of granulomatous interstitial nephritis secondary to AdV infection in a renal transplant recipient that was initially interpreted as acute graft rejection on histopathology. Specific testing based on clinical suspicion, however, aided in making an accurate diagnosis. We present a retrospective review of all cases of AdV infection in renal transplant recipients to date, and analyze outcomes based on different treatment modalities for this disease.

Opportunistic Pulmonary Infections in Immunocompromised Hosts.

Infections that are typically innocuous in immunocompetent persons may cause significant disease states in immunocompromised hosts. These individuals may be immunosuppressed secondary to many different causes such as drugs, malignancy, solid-organ or hematopoietic stem cell transplantation, HIV/AIDS, or anatomic reasons (asplenia). These immunocompromised hosts are at high risk for developing opportunistic infections. Here, we discuss some of these infections caused by bacteria, fungi, viruses, and parasites. Clinicians should be aware of the risk factors, common clinical presentations, diagnostic modalities, and treatment options for these potentially fatal illnesses.