Cardiovascular Pathology : the Official Journal of the Society for Cardiovascular Pathology

This case report presents a 20-year-old male patient initially diagnosed with infective endocarditis, later correctly identified as Behçet's syndrome. The patient's complex clinical presentation, including chest pain, aortic dilation, severe aortic regurgitation, and aortic root abscess, posed significant diagnostic and therapeutic challenges. Despite initial misdiagnosis and treatment difficulties, the patient's condition significantly improved with appropriate immunosuppressive therapy, underscoring the potential for successful management of this complex condition...

BACKGROUND: IgG4-related disease (IgG4-RD) is a recently recognized fibro-inflammatory disorder that can affect almost any organ. IgG4-RD has also been reported in coronary arteries as periarteritis. IgG4-related coronary periarteritis may cause coronary artery aneurysms, and IgG4-related coronary artery aneurysms (IGCAs) are life-threatening. We describe a case of a patient with IGCA that highlights the usefulness and limitations of various IGCA evaluation modalities and provides insight into disease pathophysiology...

Exertional dyspnea has been documented in U.S. military personnel after deployment to Iraq and Afghanistan. We studied whether continued exertional dyspnea in this patient population is associated with pulmonary vascular disease (PVD). We performed detailed histomorphometry of pulmonary vasculature in 52 Veterans with biopsy-proven post-deployment respiratory syndrome (PDRS) and then recruited five of these same Veterans with continued exertional dyspnea to undergo a follow-up clinical evaluation, including symptom questionnaire, pulmonary function testing, surface echocardiography, and right heart catheterization (RHC)...

We present a rare pediatric case of cardiac inflammatory pseudotumor (IPT) with a unique presentation of fever of unknown origin with markedly elevated inflammatory markers. A right atrial mass was discovered incidentally by echocardiography. The cardiac magnetic resonance (CMR) signal characteristics and mass location were not consistent with any of the common benign cardiac tumors of childhood. The presence of high signal intensity on T2 imaging and late gadolinium enhancement, in conjunction with intense metabolic activity at the mass site on positron emission tomography (PET), raised the possibility of an inflammatory or malignant mass...

No abstract text is available yet for this article.

BACKGROUND: In patients with aortic dissection, the aortic wall is separated into two layers along a dissection plane. In this study, a survey was performed to investigate the distribution of the depth of dissection plane and its correlation with other clinical and pathological parameters to help understand and expand the current knowledge of aortic dissection. METHODS: Pathology information system were searched for patients with aortic dissection who had undergone aortic replacement between 2019 and 2022 in Wuhan Asia General Hospital...

Quadricuspid pulmonic valve (QPV) is a rare congenital abnormality and because of its difficult non-invasive assessment, it is usually discovered incidentally at autopsies (reported prevalence in post-mortem specimens ranges from 1 in 400 to 1 in 2000) [1,12]. Unlike a bicuspid pulmonary valve, it rarely presents with clinical complications, such as valvular insufficiency or stenosis [3]. Abnormal function is rarely reported in cases that are not associated with other congenital heart disease. With increased sophistication of imaging coincidental quadricuspid valves autopsy studies are important to understand the anatomical consequences of this finding...

Cardiac fibrosis is a significant contributor to heart failure, a condition that continues to affect a growing number of patients worldwide. Various cardiovascular comorbidities can exacerbate cardiac fibrosis. While fibroblasts are believed to be the primary cell type underlying fibrosis, recent and emerging data suggest that other cell types can also potentiate or expedite fibrotic processes. Over the past few decades, clinicians have developed therapeutics that can blunt the development and progression of cardiac fibrosis...

Maternal autoimmune disease is the most common cause of congenital heart block (CHB), a rare illness characterized by fibrosis and calcification of the fetal atrioventricular (AV) node due to maternal autoantibodies anti-SSA/Ro and anti-SSB/La. We report the full autopsy and clinical information on a female neonate with high degree AV block and calcification in the AV node, atrial approaches to the AV node, and both right and left bundle branches, born to a 27-year-old female with subclinical autoimmune disease...

PURPOSE: Cardiac myxomas (CMs) are the second most common benign primary cardiac tumors, mainly originating within the left atrium. Approximately 5% of CM cases are associated with Carney Complex (CNC), an autosomal dominant multiple neoplasia syndrome often caused by germline mutations in the Protein Kinase A regulatory subunit 1A (PRKAR1A). Data concerning PRKAR1A alterations in sporadic myxomas are variable and sparse, with PRKAR1A mutations reported to range from 0% to 87%. Therefore, we investigated the frequency of PRKAR1A mutations in sporadic CM using next-generation sequencing (NGS)...

A female neonate born with normal Apgar scores at 38+2 weeks of gestational age unexpectedly passed away within less than 30 hours after birth. The situation mirrors her brother's earlier demise within 24 hours post-delivery, suggesting a possible genetic disorder. A gross examination revealed widespread cyanosis and distinct yellowish changes on the cardiac ventricles. Histopathological analysis disclosed lipid accumulation in the liver, heart, and kidney. Tandem mass spectrometry detected elevated levels of 10 amino acids and 14 carnitines in cardiac blood...

Left ventricular noncompaction (LVNC), involving mainly the right ventricle, is a rare form of congenital heart disorder characterized by a developmental arrest in myocardial compaction, resulting in a spongy appearance of the myocardium, mainly of the right ventricle, rarely detected in fetuses. We report the case of a female fetus with a gestational age of 41+4 weeks who came to our attention for intrapartum sudden unexpected death, resulting in stillbirth. The ventricular walls, particularly the right ventricular wall, appeared thick, hypertrabeculated and spongy, leading to the diagnosis of LVNC involving mainly the right ventricle...

Cardiac papillary fibroelastomas (CPFs) are rare benign cardiac tumors more often involving the left-sided valves and related with threatening embolic complications. We report the case of a 35-year-old woman presenting with relapsing-remitting chest pain and elevated cardiac troponins. After a negative coronary angiography, an integrated imaging assessment based on echocardiography and cardiac magnetic resonance showed a pedunculated mass on the aortic valve causing an intermittent obstructive engagement of the right coronary ostium...

BACKGROUND: Abdominal aortic aneurysm (AAA) is a weakening and expansion of the abdominal aorta. Currently, there is no drug treatment to limit AAA growth. The glycocalyx (GC) is the outermost layer of the cell surface, mainly composed of glycosaminoglycans (GAGs) and proteoglycans. OBJECTIVE: The aim of this review was to identify a potential relationship between GC disruption and AAA pathogenesis. METHODS: A narrative review of relevant published research was conducted...

Iatrogenic damage to the cardiac conduction system (CCS) remains a significant risk during congenital heart surgery. Current surgical best practice involves using superficial anatomical landmarks to locate and avoid damaging the CCS. Prior work indicates inherent variability in the anatomy of the CCS and supporting tissues. This study introduces high-resolution, 3D models of the CCS in normal pediatric human hearts to evaluate variability in the nodes and surrounding structures. Human pediatric hearts were obtained with an average donor age of 2...

A case of a 40-year-old male patient with a right subclavian artery aneurysm of fibromuscular dysplasia origin is reported. The patient presented with thoracic outlet-like symptoms and underwent aneurysm resection. Microscopic examination revealed intimal and medial fibroplasia. Additional cases of fibromuscular dysplasia at this rare location are reviewed, indicating a male and right-sided predominance. The most frequent clinicopathological manifestation was an aneurysm, with the histopathological pattern characterized by medial fibroplasia...

Erdheim-Chester disease (ECD) is a rare histiocytosis characterized by the foamy CD68+CD1a- histiocytes infiltrating multiple organs and tissues. ECD might be asymptomatic or present with variable manifestations. The diagnosis of ECD requires characteristic radiological findings and pathological features. Herein, we described a 52-year-old female patient who was admitted to our hospital for recurrent pericardial effusion for two months. She has a medical history of papillary thyroid carcinoma (PTC) and underwent a total thyroidectomy two years before admission...

This study aimed to assess the frequency and association between transthyretin-derived (ATTR) amyloidosis and sarcoidosis in a large autopsy cohort including many cases of sudden cardiac death (SCD). We identified 73 sporadic ATTR amyloidosis cases and 11 sarcoidosis cases, among which we found two cases with concomitant ATTR amyloidosis and sarcoidosis (2.4% of all cases; 2.7% within the sporadic ATTR group). The first case involved a 92-year-old man who experienced SCD. In this patient's heart, we observed ATTR deposition and noncaseating epithelioid granulomas consistent with sarcoidosis...

This article presents a unique case of primary myxofibrosarcoma, an exceptionally rare cardiac neoplasm, in a 16-year-old girl. With fewer than 40 documented cases, this tumor's occurrence in adolescence is particularly uncommon. The patient presented with right-sided paraesthesia and subsequent right hemiparesis and dysarthria, along with bilateral pleural effusion. Imaging tests revealed a cerebral ischemic lesion and the presence of a left atrial mass. Initially suspected as a myxoma, the tumor exhibited significant infiltration...

No abstract text is available yet for this article.