Pulmonary tumor thrombotic microangiopathy (PTTM ) is a rare condition of uncertain incidence given its likely underdiagnosis. PTTM has been described most frequently in association with gastric adenocarcinoma, but other primary malignancies have been identified. The prognosis of PTTM is very poor, and patients often die within days or weeks of diagnosis. There are, however, several medications currently being used with unknown therapeutic benefits. The case presented below describes a patient with PTTM and esophageal adenocarcinoma, which may be the first report of its kind. One other case of esophageal cancer associated with PTTM was found in the literature review, but it is of squamous cell carcinoma histology. Herein, we report a case of a male with rapidly progressive pulmonary hypertension and right heart failure who, in the course of treatment/evaluation, was found to have esophageal adenocarcinoma. While early diagnosis may not alter the course of the disease, antemortem diagnosis may identify better therapeutic options and better inform patients of their prognosis, allowing them to maintain autonomy in their medical decisions.

UNASSIGNED: Because eosinophilic granulomatosis with polyangiitis (EGPA) is so rare and the symptoms so varied, it can be a challenge to get a correct diagnosis in clinical practice. Cardiovascular involvement is the main cause of death of EGPA. We are the first to report of cardiac magnetic resonance (CMR) findings about right-sided heart involvement in EGPA.
UNASSIGNED: The initial abnormalities detected by CMR were Löffler endocarditis with extensive thrombosis and left ventricular (LV) dysfunction. After active treatment, LV systolic function recovered and endocarditis with thrombosis significantly improved, but there was rapidly progressive pulmonary hypertension, enlargement of right atrium and right ventricle and persistent right-sided heart failure. The patient eventually died of sudden cardiac death 6 months after hospital discharge.
UNASSIGNED: Löffler endocarditis and right-sided heart involvement are both rare presentations in patients with EGPA. CMR is a reliable non-invasive tool to precisely and comprehensively assess cardiovascular involvement in EGPA.

Cor triatriatum dexter (CTD) is a rare congenital heart disease resulting from persistence of the right valve of the sinus venosus. The persistent valve forms a membrane that divides the right atrium into a proximal and a distal chamber. This disorder exhibits varying clinical manifestations depending on the degree of partitioning or septation of the right atrium. In asymptomatic patients, the disease may be discovered during surgical procedures, diagnostic testing such as echocardiography, or hemodynamic monitoring. Severe septation abnormalities may cause right-sided heart failure and elevated central venous pressures due to obstruction of the tricuspid valve, right ventricular outflow tract, or inferior vena cava. Here we report a case of CTD in a patient presenting with symptoms of dyspnea and fatigue, followed by a short discussion of the embryology and clinical implications of this congenital disease, as well as current advances in management.

Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension (PH) of unknown etiology resulting from pulmonary capillary proliferation. Clinically, PCH is seen in young adults with equal sex predilection and rarely reported familial predisposition. PCH\'s main clinical presentations are progressive dyspnea, fatigue, hemoptysis, palpitations, and later irreversible pulmonary hypertension and right-sided heart failure. Hereby, we report three PCH cases, each case presented with a peculiar presentation with a comprehensive literature review highlighting etiology, clinical presentations, diagnostic modalities and pathology in establishing a diagnosis, current treatment options, and prognosis of PCH. In conclusion, defining PCH as the underlying cause of PH is of utmost importance as most medications used for PH are ineffective in PCH. Vasodilators should be avoided due to the increased risk of pulmonary oedema. Pathological examination of the lung is still considered the most definitive diagnostic tool, yet it is associated with complications risk. High-Resolution Computed Tomography (HRCT) chest is currently considered the cornerstone non-invasive modality for the diagnosis of PH. So far, no definitive treatment of PCH excluding lung transplantation with preliminary promising results with angiogenesis Inhibitors. PCH carries a very poor prognosis with a median survival of 3 years from the time of diagnosis.

We herein report the case of a 79-year-old man who presented with right-sided heart failure (HF) 27 years after undergoing surgery for tetralogy of Fallot. The HF did not respond well to oral diuretics. Transthoracic echocardiography and chest X-ray failed to determine the cause of the HF for three years. An intrapericardial mass located just behind the sternum, was finally identified on computed tomography. The mass had compressed the right ventricle, causing right-sided HF. Pre-surgical diagnostic images led to suspicion of a chronic expanding intrapericardial hematoma (CEIH), and the CEIH was surgically removed. The patient\'s symptoms improved markedly.

UNASSIGNED: Constrictive pericarditis (CP) is a pathological condition of the pericardium, resulting from fibrosis, scarring, and calcification of the pericardium. Other conditions have been reported to mimic \'constrictive physiology\' despite the presence of an intact pericardium. However, there has been no report of pulmonary regurgitation (PR) mimicking the haemodynamic characteristics of CP.
UNASSIGNED: A 51-year-old woman was admitted to our institute because of severe right-sided heart failure. Transthoracic echocardiography revealed severe PR concomitant with significant dilatation of the right-sided heart. Septal bounce and the respiratory reciprocation of the transmitral and transtricuspid inflow velocities were also observed, indicating exacerbated ventricular interdependence. Cardiac catheter examination demonstrated elevated right atrial pressure with a prominent y descent, dip, and plateau waveform in the right ventricular pressure, and equalization of the diastolic pressure of all cardiac chambers, which are quite consistent with CP. On surgical inspection, however, there was no pericardial thickening or adhesion, indicating no obvious signs of CP.
UNASSIGNED: Pericardial constriction results from the relative relationship between intrapericardial volume and pericardial reserve. When the intrapericardial volume exceeds the physiological limit, the cardiac chambers compete with each other in a fixed pericardial space. In this case, prominent dilation of the right-sided chambers caused by severe PR resulted in overstretching of the pericardium above the pericardial reserve, which led to a characteristic haemodynamic picture that resembled CP. Thus, it is important to recognize the diagnostic pitfall in the preoperative evaluation of a \'CP mimic physiology\'.

Klippel-Trenaunay syndrome is a rare congenital mesodermal abnormality characterized by varicose veins, cutaneous hemangiomas, soft tissue and bony hypertrophy of limb. Potential complications such as deep venous thrombosis and pulmonary thromboembolism have not been reported in Korea to date. We demonstrate the case of a 48-year-old woman with Klippel-Trenaunay syndrome with extensive varicose veins on right lower limb, hypertrophy of left big toe and basilar artery tip aneurysm, complicated with acute submassive pulmonary thromboembolism treated successfully with intravenous thrombolytic therapy.

Arterial leiomyosarcomas account for up to 21% of vascular leiomyosarcomas, with 56% of arterial leiomyosarcomas occurring in the pulmonary artery. While isolated cases of primary pulmonary artery leiomyosarcoma document survival up to 36 months after treatment, these uncommon, aggressive tumors are highly lethal, with 1-year survival estimated at 20% from the onset of symptoms. We discuss a rare case of a pulmonary artery leiomyosarcoma that was originally diagnosed as a pulmonary embolism (PE). A 72-year-old Caucasian female was initially diagnosed with \'saddle pulmonary embolism\' based on computerized tomographic angiography of the chest 2 months prior to admission and placed on anticoagulation. Dyspnea escalated, and serial computed tomography scans showed cardiomegaly with pulmonary emboli involving the right and left main pulmonary arteries with extension into the right and left upper and lower lobe branches. An echocardiogram on admission showed severe pulmonary hypertension with a pulmonary artery pressure of 82.9 mm Hg, and a severely enlarged right ventricle. Respiratory distress and multiorgan failure developed and, unfortunately, the patient expired. Autopsy showed a lobulated, yellow mass throughout the main pulmonary arteries measuring 13 cm in diameter. The mass extended into the parenchyma of the right upper lobe. On microscopy, the mass was consistent with a high-grade primary pulmonary artery leiomyosarcoma. Median survival of patients with primary pulmonary artery leiomyosarcoma without surgery is one and a half months, and mortality is usually due to right-sided heart failure. Pulmonary artery leiomyosarcoma is a rare but highly lethal disease commonly mistaken for PE. Thus, we recommend clinicians to suspect this malignancy when anticoagulation fails to relieve initial symptoms. In conclusion, early detection and suspicion of pulmonary artery leiomyosarcoma should be considered in patients refractory to anticoagulation, prompting initiation of early intervention.