Serum PM20D1 levels in patients with idiopathic pulmonary arterial hypertension and its clinical significance.

OBJECTIVE: This study aimed to investigate the serum levels of Peptidase M20 domain containing 1 (PM20D1) in idiopathic pulmonary arterial hypertension (IPAH) patients and examine its association with lipid metabolism, echocardiography, and hemodynamic parameters. METHODS: This prospective observational research enrolled 103 IPAH patients from January 2018 to January 2022. Enzyme-linked immunosorbent assay (ELISA) was used to measure the serum PM20D1 levels in all patients before treatment within 24 h of admission. Demographic data, echocardiography, hemodynamic parameters and serum biomarkers were also collected. RESULTS: The IPAH patients in the deceased group had significantly elevated age, right atrial (RA), mean pulmonary arterial pressure (mPAP), mean right atrial pressure (mRAP), pulmonary capillary wedge pressure (PCWP), pulmonary vascular resistance (PVR) and significantly decreased 6 min walking distance (6MWD) and tricuspid annulus peak systolic velocity (TASPV). IPAH patients showed significant decreases in serum PM20D1, low-density lipoprotein cholesterol (LDL-C), and albumin (ALB). Additionally, PM20D1 was negatively correlated with RA, NT-proBNP and positively correlated with PVR, ALB, 6MWD, and TAPSV. Moreover, PM20D1 has the potential as a biomarker for predicting IPAH patients' prognosis. Finally, logistic regression analysis indicated that PM20D1, ALB, NT-proBNP, PVR, TASPV, RA and 6MWD were identified as risk factors for mortality in IPAH patients. CONCLUSION: Our findings indicated that the serum levels of PM20D1 were significantly decreased in IPAH patients with poor prognosis. Moreover, PM20D1 was identified as a risk factor associated with mortality in IPAH patients.

All that Doesn't Enhance Isn't a Thrombus: Pitfalls Using Cardiac MRI TI 600 Sequence to Distinguish Between Cardiac Thrombus Versus Myxoma.

A 51-year-old male with a complicated medical history presented with shortness of breath. Preoperative workup confirmed the presence of a large atrial mass. However, delayed gadolinium enhancement CMR with long inversion time (TI 600) showed lack of enhancement, which was suggestive of a thrombus. During cardiac magnetic resonance imaging, delayed gadolinium enhancement sequences with long inversion time (TI 600) are commonly used to distinguish between an avascular thrombus versus a vascular tumor.

Swiss Cheese Heart: A Tale of Multiple Valve Perforations.

Gerbode defect, an anomalous connection between the left ventricle and right atrium, is often congenital but can be acquired or iatrogenically formed. We present an exceedingly rare case of this defect associated with multiple valve perforation in an otherwise healthy patient with bicuspid aortic valve and endocarditis.

Uncommon using of the pulmonary homograft in oncological case - three years follow up.

INTRODUCTION: There are enough cases of colorectal cancer with liver metastasis, but inferior vena cava infiltraion with dissemination to the right atrium is an infrequent event. PRESENTATION OF CASE: This is the first case of surgical treatment of recurrent liver metastasis with the infiltration to the inferior vena cava and to the right atrium of the heart, using a cryopreserved pulmonary homograft. DISCUSSION: The choice of a cryopreserved pulmonary homograft was preferred by the need for a radical and wide resection of tissues involved in the metastasis, as well as to potentially reduce the risk of thrombosis in the short- and long-term postoperative period. CONCLUSION: The use of a cryopreserved homograft in operation undergoing cardiopulmonary bypass allowed us to perform the required volume of radical resection and to replace an extended section of the inferior vena cava.

Combination of electrophysiological mapping, radiofrequency catheter ablation, and atrial appendectomy in a 5-year-old girl with tachycardia-induced cardiomyopathy: a case report.

BACKGROUND: Atrial tachycardia (AT) originating from the left atrial appendage (LAA) is uncommon and the most difficult arrhythmia to eliminate. Therefore, we present the case of a 5-year-old girl with tachycardia-induced cardiomyopathy (TIC) caused by AT originating from the LAA and successfully treated with RFCA associated to left atrial appendectomy. With resolution of AT, we observed a progressive improvement of LV function. The effectiveness and safety of this combination therapy were evaluated over a one-month follow-up period. CASE PRESENTATION: A 5 -year-old female was evaluated for three days of incessant cough and a syncopal episode. Surface echocardiography and 24-hour monitoring showed that the infant had persistent atrial tachycardia. Echocardiography revealed an enlarged tele diastolic diameter (46.1 mm) and malfunctioning (EF 28.53%) left ventricle. The location of the lesion at the apex of the LAA was further confirmed by electrophysiological study and RFCA. After RFCA, the infant's ECG monitor showed that sinus rhythm was maintained for up to 22 h. Subsequently, atrial tachycardia recurred and sinus rhythm disappeared. Finally, atrial appendectomy was performed and sinus rhythm returned to normal. CONCLUSIONS: The heart function of the infant improved and sinus rhythm was maintained, further demonstrating the safety and effectiveness of combined treatment with RFCA and atrial appendectomy after electrophysiological localization of AT from LAA to TIC.

Periodontal Treatment During the Blanking Period Improves the Outcome of Atrial Fibrillation Ablation.

BACKGROUND: Periodontitis has not been recognized as a modifiable risk factor for atrial fibrillation (AF). This prospective nonrandomized study investigated whether periodontal treatment improves the AF ablation outcome. METHODS AND RESULTS: We prospectively enrolled 288 AF patients scheduled to undergo initial radiofrequency catheter ablation. Each patient underwent periodontal inflamed surface area (PISA; a quantitative index of periodontal inflammation) measurement. All eligible patients were recommended to receive periodontal treatment within the blanking period, and 97 consented. During the mean follow-up period of 507±256 days, 70 (24%) AF recurrences were documented. Patients who exhibited AF recurrences had a higher PISA than those who did not (456.8±403.5 versus 277.7±259.0 mm2, P=0.001). These patients were categorized into high-PISA (>615 mm2) and low-PISA (<615 mm2) groups according to the receiver operating characteristic analysis for AF recurrence (area under the curve, 0.611; sensitivity, 39%; specificity, 89%). A high PISA, as well as female sex, AF duration, and left atrial volume, were the statistically significant predicter for AF recurrence (hazard ratio [HR], 2.308 [95% CI, 1.234-4.315]; P=0.009). In patients with a high PISA, those who underwent periodontal treatment showed significantly fewer AF recurrences (P=0.01, log-rank test). The adjusted HR of periodontal treatment for AF recurrence was 0.393 (95% CI, 0.215-0.719; P=0.002). CONCLUSIONS: Periodontitis may serve as a modifiable risk factor for AF. PISA is a hallmark of AF recurrence, and periodontal treatment improves the AF ablation outcome, especially for those with poor periodontal condition.

Surgical strategy for intravenous leiomyomatosis spreading from uterine to the right atrium presenting with recurrent syncope.

Uterine leiomyoma invading internal iliac vein and consequently disseminating into the right atrium is an extremely rare condition, and surgical strategy is controversial. Here, we reported a specific case with successful surgical resection through one-stage total hysterectomy, bilateral oophorectomy, and the intracardiovascular lesion. This procedure would be an optimal choice for uterine leiomyoma invading inferior vena cava and spreading to right atrium.

Multiple thrombi mimicking metastases in the right atrium of patients with non-Hodgkin's lymphoma diagnosed by multimodal cardiac imaging: one case report.

BACKGROUND: Right-side heart mass can be found incidentally on routine transthoracic echocardiography (TTE). Accurate diagnosis of cardiac mass often requires more than one imaging method. We present a mid-age woman with non-Hodgkin lymphoma who was found to have multiple right atrial masses mimicking metastases on routine TTE, which were finally diagnosed as thrombi by multimodal cardiac imaging. CASE PRESENTATION: A 52-year-old woman was diagnosed with primary mediastinal diffuse large B cell lymphoma (DLBCL) almost six months prior. The TTE revealed multiple masses in the right atrium with normal cardiac function when she was being evaluated for the next chemotherapy. On arrival, she was hemodynamically stable and asymptomatic. Physical examination was no remarkable. Laboratory findings showed leukocytosis of 17,900 cells/mm3, hemoglobin of 7.5 mg/dL, and a normal D-dimer level. The suspicious diagnosis of right atrial metastasis was made by TEE. However, the diagnosis of right atrial thrombi was made by contrast CMR. Finally, the 18 F-FDG PET-CT demonstrated no metabolic activity in the right atrium, which further supported the diagnosis of thrombi. Eventually, the masses were removed by cardiopulmonary bypass thoracotomy because of a high risk of pulmonary embolism. Histopathology confirmed the diagnosis of thrombi. CONCLUSIONS: This case highlights the importance of multimodality cardiac imaging in the appropriate diagnosis of a RA masses in patient of lymphoma. Diagnosis of RA masses can be made using multimodal cardiac imaging like TTE, TEE and CMR, even PET. Echocardiography is the most commonly used on multimodal imaging in cardiac thrombus. CMR has high specificity in differentiating a tumor from thrombus, while 18 F-FDG PET has good sensitivity to determine the nature of the masses.

A rare presentation of primary cardiac myxofibrosarcoma: Case report and literature review.

BACKGROUND: Primary cardiac myxofibrosarcoma is a rare and aggressive malignancy, with the majority of approaching strategies relying on case reports. This article provides insights into its diagnosis and treatment. CASE PRESENTATION: This paper presents the case of a 40-year-old man with sudden onset hemoptysis, leading to the diagnosis of primary cardiac myxofibrosarcoma. Treatment involved open-heart surgery to excise the left atrium tumor, followed by 6 cycles of adjuvant chemotherapy. Unfortunately, brain metastasis developed, leading to the patient's death 1 year after initial diagnosis. CONCLUSION: Primary cardiac myxofibrosarcoma remains a clinical challenge with an unfavorable prognosis. Early diagnosis through advanced imaging is crucial, and research is needed to explore innovative treatments. This case underscores the complexities of managing this rare cardiac malignancy and highlights the necessity for ongoing investigations to enhance patient outcomes.

An Increase in the Left Atrium Volume During Exercise is Associated With a Positive Result of a Diastolic Stress Test in Patients With Arterial Hypertension.

AIM: To study the relationship between changes in left atrial volume (LAV) during exercise and the result of a diastolic stress test (DST) in patients with arterial hypertension (AH). MATERIAL AND METHODS: The study included 219 patients with AH without ischemic heart disease and atrial fibrillation. During the DST performed before and after exercise, the ratio of transmitral flow velocity to mitral annular velocity (E/e'), the left atrial global longitudinal strain in the reservoir phase (reservoir strain), and LAV were determined. The criterion for a positive DST was an increase in E/e' ≥15. RESULTS: A positive result of DST was observed in 90 (41.1%) patients. Patients with positive DST were older (65.0 and 59.0 years); among them, there were fewer men (24.4 and 41.1%), but more patients with obesity (66.7 and 40.3%) and diabetes mellitus (36.7 and 8.5%). At rest, patients with positive DST had higher E/e' ratio (11.5 and 8.8), pulmonary artery systolic pressure (29.0 and 27.0 mm Hg), and LAV (60.0 and 52.0 ml), but a lower left atrial reservoir strain (20.0 and 24.0%). During exercise in patients with positive and negative DST, E/e' increased by 5.46 and 0.47 units, respectively. Changes in the LAV and reservoir strain during exercise in these groups were directed differently. In patients with positive DST, the left atrial reservoir strain decreased by 1.0 percentage points (pp) whereas in patients with negative DST, it increased by 8.0 pp. During exercise, the LAV increased by 10.0 ml in patients with a positive DST, whereas in the alternative group, the LAV decreased by 8.5 ml. The AUC for changes in LAV as an indicator of a positive DST was 0.987 while the AUC for the resting left atrial reservoir strain was 0.938. An increase in LAV >1 ml, as an indicator of a positive DST has a sensitivity of 96.9% and a specificity of 95.1%. CONCLUSION: In AH patients, changes in left ventricular filling pressure are associated with a unidirectional change in LAV. An increase in LAV during exercise by more than 1 ml can serve as a criterion for a positive DST result. This assessment was consistent with the assessment of the DST result by the E/e' criterion >15 in 94.5% of cases.

Determining The Risk of Atrial Fibrillation Paroxysm in Patients With Chronic Heart Failure With Intact and Reduced Ejection Fraction.

AIM: To determine predictors for the development of atrial fibrillation (AF) in patients with chronic heart failure (CHF) with preserved and reduced ejection fraction by echocardiography (EchoCG) according to an extended protocol with determination of diastolic function and left atrial global strain. MATERIAL AND METHODS: Data of 168 patients with stage I-III CHF without a history of AF were analyzed. All patients underwent echocardiography according to an extended protocol with the determination of diastolic dysfunction (DD), left atrial ejection fraction (LA EF), and left atrial global strain (LA GS). Tissue Doppler imaging (TDI) was used to evaluate the early (E) and late (A) LV filling velocity and the early (E') and late (A') diastolic mitral annular velocity. In all patients, Holter ECG monitoring (HM ECG) of heart rhythm was performed for 3 days, and ECG monitoring with telemedicine technologies was performed for 7 days, 3 times a day for 3 minutes. The follow-up period was 3 months or until an AF episode. RESULTS: During the study, paroxysmal AF (pAF) was detected in 41 (24.4%) patients using various methods of heart rhythm monitoring. Complaints of palpitations were noted for 10 (24.4%) patients during pAF, which was recorded using a CardioQVARK® device, HM ECG or a 12-lead ECG. In 5 (12.2%) patients, daily ECG monitoring revealed pAF without associated complaints. HM ECG detected 8, 2, 4 (19.5%, 4.8%, and 9.7%) cases during 24, 48 and 72 hours, respectively; a single-channel CardioQVARK® detected 30 (73.2%) cases when used 3 times a day for 7 days. These results showed that AF frequently develops in CHF without accompanying symptoms. The method for detecting pAF with CardioQVARK® showed good results: it was twice more effective than HM ECG and three times more effective than 12-lead ECG. Also, according to ultrasound data, significant changes in the following parameters were noted in patients with AF: LA EF <36% (OR 1.04, 95% CI: 1.02-1.08), p=0.003; LA GS <9.9% (OR 1.16, 95% CI: 1.02-1.38), p<0.001; TDI E med <5.7 cm/s (OR 0.97, 95% CI: 0.94-1.00), p=0.026. Grade 2 DD did not show statistically significant results (OR 1.1, 95% CI: 0.7-1.5, p=0.54). However, it was detected more frequently in patients with AF, in 34% of cases, compared to 29% of cases in patients without AF, which requires further study on a larger patient sample. CONCLUSION: Patients with CHF have a high risk of developing pAF (24.4%). 75% of patients with AF do not feel the development of paroxysm. All CHF patients should undergo EchoCG with assessment of LA EF, TDI E med and LA GS to identify a group at risk for the development of AF. Heart rhythm remote monitoring with CardioQVARK® devices can be considered a reliable method for early detection of pAF and timely initiation of anticoagulant therapy in patients with CHF.

Assessing emerging causes of mitral regurgitation: atrial functional mitral regurgitation.

Functional or secondary mitral regurgitation is linked to increased cardiovascular morbidity and mortality. From a mechanical perspective, secondary mitral regurgitation occurs due to an imbalance between the forces that tether the mitral leaflets and those that close them. This results in incomplete coaptation. Most commonly, functional mitral regurgitation, which occurs in both ischaemic and non-ischaemic disease states, is usually caused by dysfunction and changes in the left ventricle. Atrial functional mitral regurgitation (AFMR) is a disease state that has been more recently recognized. It occurs when mitral annular enlargement is associated with left atrial dilatation, preserving left ventricular geometry and function. AFMR is typically seen in patients with chronic atrial fibrillation or heart failure who have a conserved ejection fraction. Published reports and ongoing investigations vary in how they define AFMR. This publication examines the pathophysiology of AFMR and highlights the importance of having a common working standard for the definition of AFMR to ensure consistency in the data reported and to drive forward the much needed research into the outcomes and treatment strategies in this area. Several studies have reported that restrictive annuloplasty and transcatheter edge-to-edge repair can reduce mitral regurgitation and improve symptoms. This narrative review will explore the pathophysiology, echocardiographic diagnosis and treatment of AFMR.

Impact of atrial fibrillation phenotype and left atrial volume on outcome after pulmonary vein isolation.

AIMS: Pulmonary vein isolation (PVI) is increasingly performed in patients with atrial fibrillation (AF). Both AF phenotype and left atrial (LA) volume have been shown to influence ablation outcome. The inter-relationship of the two is incompletely understood. We aimed to investigate the impact of AF phenotype vs. LA volume on outcome after PVI. METHODS AND RESULTS: In a retrospective analysis of a prospective registry of patients undergoing a first PVI, the association of AF phenotype and LA volume index (LAVI) was assessed as well as their impact on AF recurrence during follow-up. Overall, 476 patients were enrolled (median age 63 years, 29% females, 65.8% paroxysmal AF). Obesity, hypertension, chronic kidney disease, and heart failure were all significantly more frequent in persistent AF. After 1 year, single-procedure, freedom from arrhythmia recurrence was 61.5%. Patients with paroxysmal AF had better outcomes compared with patients with persistent AF (65.6 vs. 52.7%, P = 0.003), as had patients with no/mild vs. moderate/severe LA dilation (LAVI <42 mL/m2 67.1% vs. LAVI ≥42 mL/m2 53%, P < 0.001). The combination of both parameters refined prediction of 1-year recurrence (P < 0.001). After adjustment for additional clinical risk factors in multivariable Cox proportional hazard analysis, both AF phenotype and LAVI ≥42 mL/m2 contributed significantly towards the prediction of 1-year recurrence. CONCLUSION: Atrial fibrillation phenotype and LA volume are independent predictors of outcome after PVI. Persistent AF with no/mild LA dilation has a similar risk of recurrence as paroxysmal AF with a moderate/severe LA dilation and should be given similar priority for ablation.

An interesting electrocardiogram caused by lead reversal.

BACKGROUND: During normal sinus rhythm, atrial depolarization is conducted from right atrium to left atrium through Bachmann's bundle, and a normal P wave axis which is measured on the frontal plane is between 0º and + 75º. The change of P wave polarity is helpful for the analysis of origin point. CASE PRESENTATION: We report a patient with negative P wave in lead I. The characteristics of QRS complex in leads V1 to V6 are helpful to preliminarily differential diagnosis. The 12-lead electrocardiogram (ECG) with correct limb leads (right arm-left arm) placement shows sinus rhythm with complete right bundle branch block (RBBB). CONCLUSIONS: The change of P wave polarity as well as characteristics of QRS complex can help identify limb-lead reversals.

Performance of Image-navigated and Diaphragm-navigated 3D Late Gadolinium-enhanced Cardiac MRI for the Assessment of Atrial Fibrosis.

Purpose To perform a qualitative and quantitative evaluation of the novel image-navigated (iNAV) 3D late gadolinium enhancement (LGE) cardiac MRI imaging strategy in comparison with the conventional diaphragm-navigated (dNAV) 3D LGE cardiac MRI strategy for the assessment of left atrial fibrosis in atrial fibrillation (AF). Materials and Methods In this prospective study conducted between April and September 2022, 26 consecutive participants with AF (mean age, 61 ± 11 years; 19 male) underwent both iNAV and dNAV 3D LGE cardiac MRI, with equivalent spatial resolution and timing in the cardiac cycle. Participants were randomized in the acquisition order of iNAV and dNAV. Both, iNAV-LGE and dNAV-LGE images were analyzed qualitatively using a 5-point Likert scale and quantitatively (percentage of atrial fibrosis using image intensity ratio threshold 1.2), including testing for overlap in atrial fibrosis areas by calculating Dice score. Results Acquisition time of iNAV was significantly lower compared with dNAV (4.9 ± 1.1 minutes versus 12 ± 4 minutes, P < .001, respectively). There was no evidence of a difference in image quality for all prespecified criteria between iNAV and dNAV, although dNAV was the preferred image strategy in two-thirds of cases (17/26, 65%). Quantitative assessment demonstrated that mean fibrosis scores were lower for iNAV compared with dNAV (12 ± 8% versus 20 ± 12%, P < .001). Spatial correspondence between the atrial fibrosis maps was modest (Dice similarity coefficient, 0.43 ± 0.15). Conclusion iNAV-LGE acquisition in individuals with AF was more than twice as fast as dNAV acquisition but resulted in a lower atrial fibrosis score. The differences between these two strategies might impact clinical interpretation. ©RSNA, 2024.

Bleeding sites and treatment strategies for cardiac tamponade by catheter ablation requiring thoracotomy: risks of catheter ablation in patients with left atrial diverticulum.

BACKGROUND: There is insufficient information regarding the bleeding sites and surgical strategies of cardiac tamponade during catheter ablation for atrial fibrillation (AF). CASE PRESENTATION: Of the five patients with cardiac tamponade, three required surgical intervention and two required pericardiocentesis. In the first case of three cardiac tamponades requiring surgical intervention, considering that the peripheral route was used, the catecholamines did not reach the heart, and due to unstable vital signs, venoarterial extracorporeal membrane oxygenation (VA-ECMO) was inserted. No bleeding point was identified, but a thrombus had spread around the left atrium (LA) with diverticulum. Hemostasis was achieved with adhesives placed around the LA under on-pump beating. In the second case, pericardiocentesis was performed, but the patient showed heavy bleeding and unstable vital signs. Thus, VA-ECMO was inserted. Heavy bleeding was expected, and safety was enhanced by attaching a reservoir to the VA-ECMO. The bleeding point was found between the left upper pulmonary artery and LA under cardiac arrest to obtain a good surgical view for suturing repair. In the third case, the LA diverticulum was damaged. Pericardiocentesis resulted in stable vitals, but sustained bleeding was present. A bleeding point was found at the LA diverticulum, and suture repair under on-pump beating was performed. CONCLUSIONS: When cardiac tamponade occured in any patient with LA diverticulum, treatment could not be completed with pericardiocentesis alone, and thoracotomy was likely to be necessary. If the bleeding point could be confirmed, suturing technique is a more reliable surgical strategy than adhesive alone that leads to pseudoaneurysm. If the bleeding point is unclear, it is important to confirm the occurrence of LA diverticulum using a preoperative CT, and if confirmed, cover it with adhesive due to a high possibility of diverticulum bleeding. The necessity of CPB should be determined based on whether these operations can be completed while maintaining vital stability.

Electrophysiological cardiovascular MR: procedure-ready mesh model generation for interventional guidance based on non-selective excitation compressed sensing whole heart imaging.

Fully CMR-guided electrophysiological interventions (EP-CMR) have recently been introduced but data on the optimal CMR imaging protocol are scarce. This study determined the clinical utility of 3D non-selective whole heart steady-state free precession imaging using compressed SENSE (nsWHcs) for automatic segmentation of cardiac cavities as the basis for targeted catheter navigation during EP-CMR cavo-tricuspid isthmus ablation. Fourty-two consecutive patients with isthmus-dependent right atrial flutter underwent EP-CMR radiofrequency ablations. nsWHcs succeeded in all patients (nominal scan duration, 98 ± 10 s); automatic segmentation/generation of surface meshes of right-sided cavities exhibited short computation times (16 ± 3 s) with correct delineation of right atrium, right ventricle, tricuspid annulus and coronary sinus ostium in 100%, 100%, 100% and 95%, respectively. Point-by-point ablation adhered to the predefined isthmus line in 62% of patients (26/42); activation mapping confirmed complete bidirectional isthmus block (conduction time difference, 136 ± 28 ms). nsWHcs ensured automatic and reliable 3D segmentation of targeted endoluminal cavities, multiplanar reformatting and image fusion (e.g. activation time measurements) and represented the basis for precise real-time active catheter navigation during EP-CMR ablations of isthmus-dependent right atrial flutter. Hence, nsWHcs can be considered a key component in order to advance EP-CMR towards the ultimate goal of targeted substrate-based ablation procedures.

A surgical resection case of myxoma arising from the posterior wall of the left atrium complicated with complete atrioventricular block.

An 80-year-old female was referred to our institution due to transient right upper limb weakness. Transthoracic and transesophageal echocardiography revealed a tumor in the left atrium. The tumor was attached to the posterior wall of the left atrium near the atrioventricular node. Intraoperative pathological examination revealed that the tumor was a myxoma, and complete resection was successfully performed. However, she experienced persistent complete atrioventricular block postoperatively and required pacemaker implantation.