-
Frontiers in Pediatrics 2024Massive tricuspid regurgitation (TR) is the most common feature of pulmonary atresia with intact ventricular septum (PA/IVS), and mild or absent TR is observed in severe...
BACKGROUND
Massive tricuspid regurgitation (TR) is the most common feature of pulmonary atresia with intact ventricular septum (PA/IVS), and mild or absent TR is observed in severe right ventricular (RV) dysplasia or RV-to-coronary fistulous connections, resulting in non-biventricular (BV) outcomes postnatally.
CASE SUMMARY
We report a case of fetal severe pulmonary stenosis with IVS diagnosed at 26 weeks of gestation. The severity of RV hypoplasia did not worsen or reach indications for intrauterine intervention, while the jet velocity of TR decreased significantly during pregnancy. The fetus was definitely diagnosed with PA/IVS with mild RV dysplasia after birth. Unusually, the fetus did not experience severe TR and myocardial sinusoids, the TR jet velocity was maintained at 2.0 m/s, and the coronary artery was almost normal. The incapable RV cannot pump blood into pulmonary circulation after RV decompression from valvular perforation and balloon dilation. It may be an extraordinary finding of subsystemic RV.
CONCLUSION
PA/IVS is a heterogeneous disease with various degrees of RV dysplasia. Mild or no baseline TR is a reliable indicator with non-BV outcomes for fetal PA/IVS, even with acceptable dysplasia RV structures.
PubMed: 38751746
DOI: 10.3389/fped.2024.1251274 -
JACC. Cardiovascular Interventions May 2024Transcatheter aortic valve replacement (TAVR) in patients with bicuspid aortic valve (BAV) stenosis is technically challenging and is burdened by an increased risk of...
BACKGROUND
Transcatheter aortic valve replacement (TAVR) in patients with bicuspid aortic valve (BAV) stenosis is technically challenging and is burdened by an increased risk of paravalvular regurgitation (PVR).
OBJECTIVES
To identify the incidence, predictors, and clinical outcomes of PVR following TAVR in Sievers type 1 BAV stenosis.
METHODS
Consecutive patients with severe Sievers type 1 BAV stenosis undergoing TAVR with current generation transcatheter heart valves (THVs) in 24 international centres were enrolled. PVR was graded as none/trace, mild, moderate, and severe according to echocardiographic criteria. The endpoint of major adverse events (MAE), defined as a composite of all-cause death, stroke, or hospitalization for heart failure, was assessed at the last available follow-up.
RESULTS
A total of 946 patients were enrolled. PVR occurred in 423 patients (44.7%): mild, moderate, and severe in 387 (40.9%), 32 (3.4%), and 4 (0.4%) patients, respectively. Independent predictors of moderate or severe PVR were larger virtual raphe ring (VRR) perimeter (OR 1.07, 95% CI 1.02-1.13), severe annular or left ventricular outflow tract (LVOT) calcification (OR 5.21, 95% CI 1.45-18.77), self-expanding valve (OR 9.01, 95% CI 2.09-38.86), and intentional supra-annular THV positioning (OR 3.31, 95% CI 1.04-10.54). At a median follow-up of 1.3 [IQR 0.5-2.4] years, moderate or severe PVR was associated with an increased risk of MAE (HR 2.52, 95% CI 1.24-5.09).
CONCLUSIONS
After TAVR with current-generation THVs in Sievers type 1 BAV stenosis, moderate or severe PVR occurred in about 4% of cases and was associated with an increased risk of MAE during follow-up.
PubMed: 38749449
DOI: 10.1016/j.jcin.2024.05.002 -
Journal of Cardiothoracic Surgery May 2024Pulmonary arterial sarcomas (PAS) are rare aggressive tumours occurring mainly in the pulmonary trunk. We report a case of PAS involving the pulmonary trunk wall and... (Review)
Review
Pulmonary arterial sarcomas (PAS) are rare aggressive tumours occurring mainly in the pulmonary trunk. We report a case of PAS involving the pulmonary trunk wall and valve, with uniform wall thickening which represents an atypical imaging manifestation of this tumour. A 63-year-old male presented with vague respiratory symptoms with rapid progression. CTPA showed low density filling defects in both pulmonary arteries and PET scan showed increased uptake in the pulmonary trunk, which along with raised ESR suggested Pulmonary Vasculitis. Echo imaging showed Right ventricular hypertrophy and pulmonary stenosis. Response to steroid therapy was minimal and his symptoms worsened. A referral for second opinion was made and he was diagnosed with PAS. He underwent Pulmonary thromboendarterectomy with Pulmonary valve replacement. Post-operative histopathology confirmed the diagnosis. PAS is rare and frequently misdiagnosed. Surgical resection is not curative, but together with chemotherapy can prolong survival.
Topics: Humans; Male; Middle Aged; Pulmonary Artery; Sarcoma; Pulmonary Valve; Vascular Neoplasms; Diagnosis, Differential; Vasculitis; Diagnostic Errors
PubMed: 38745263
DOI: 10.1186/s13019-024-02700-3 -
International Medical Case Reports... 2024SARS-CoV-2 virus has led to an unprecedented amount of tracheal stenosis. Rigid bronchoscopy can serve as a curative measure or bridge therapy to tracheal resection. We...
SARS-CoV-2 virus has led to an unprecedented amount of tracheal stenosis. Rigid bronchoscopy can serve as a curative measure or bridge therapy to tracheal resection. We also briefly discuss the pathophysiology of tracheal stenosis from prolonged intubation and SARS-CoV-2 virus. This should be differentiated from other forms of airway obstruction such as tracheobronchomalacia which would be considered a pseudo-tracheal stenotic disease. The aim of this study is to evaluate stenosis that is unable to be improved with positive airway pressure or "PAP" therapies and required stenting and/or subsequent tracheal resection. By performing Rigid Bronchoscopy and subsequent stenting of airways, we demonstrated outcomes for long term airway patency regarding patients who were intubated secondary to the SARS-CoV-2 virus. We demonstrate superb outcomes in a consecutive case series of 6 patients managed with rigid bronchoscopy, airway stent and tracheal resection. The patients were all managed from a pulmonary perspective by the physicians mentioned in this study.
PubMed: 38737213
DOI: 10.2147/IMCRJ.S436903 -
Journal of Cardiothoracic Surgery May 2024Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease characterized by the inability of all pulmonary veins to connect to the left...
BACKGROUND
Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease characterized by the inability of all pulmonary veins to connect to the left atrium. Our previous bibliometric article summarized the characteristics of only the 100 most cited papers in TAPVC research. The purpose of this study was to use comprehensive bibliometric analysis to examine the development history, current status, and future trends in the field of TAPVC.
METHODS
All publications on TAPVC published between 2000 and 2023 were collected from the Web of Science Core Collection. The publication and citation data were quantitatively analyzed by publication year, country, institution, author, and journal. Co-authorship and co-occurrence analyses were performed using VOSviewer, and keyword and reference bursts were identified using CiteSpace. Pearson's test was used to examine the correlations between two continuous variables.
RESULTS
As of July 20, 2023, we identified 368 publications with 3320 citations. These publications were published in 132 journals and authored by 1835 researchers from 457 institutions in 47 countries. For the number of publications, the top country, top institution, top author, and top journals were the United States (n = 82), Shanghai Jiao Tong University (n = 13), Huiwen Chen (n = 9), and Annals of Thoracic Surgery and Pediatric Cardiology (n = 29 each), respectively. For the number of citations, the top country, top affiliation, top author, and top journal were the United States (n = 1348), University of Toronto (n = 250), Christopher A. Caldarone (n = 315), and Annals of Thoracic Surgery (n = 746), respectively. The number of national publications significantly correlated with GDP (R = 0.887, P < 0.001), research & development (R&D) expenditure (R = 0.375, P = 0.013), population (R = 0.694, P < 0.001), and journals (R = 0.751, P < 0.001). The number of national citations significantly correlated with GDP (R = 0.881, P < 0.001), R&D expenditure (R = 0.446, P = 0.003), population (R = 0.305, P = 0.037), and journals (R = 0.917, P < 0.001). International collaboration in the field of TAPVC was not well developed. The most commonly cited publication discussed era changes in mortality and reoperation rate in TAPVC patients. The most common keywords were "total anomalous pulmonary venous connection" and "congenital heart disease". The keyword "case report" appeared most recently, with an average occurrence year of 2021.8. The co-occurrence analysis grouped 26 keywords into six themes: surgical repair of TAPVC, postoperative pulmonary vein stenosis, surgical repair of TAPVC patients with heterotaxy, application of echocardiography in diagnosing TAPVC, application of echocardiography in the prenatal diagnosis of TAPVC, and application of the sutureless technique in the surgical repair of TAPVC patients with right atrial isomerism or a single ventricle. Citation burst detection identified 32 references with citation bursts, seven of which had ongoing citation bursts until 2023.
CONCLUSIONS
This study conducted a bibliometric analysis to provide a comprehensive overview of TAPVC research. We hope to offer new ideas for promoting development in the field of TAPVC.
Topics: Bibliometrics; Humans; Scimitar Syndrome; Biomedical Research
PubMed: 38730414
DOI: 10.1186/s13019-024-02787-8 -
Annals of Cardiac Anaesthesia Jan 2024The occurrence of pulmonary artery thrombus in association with rheumatic mitral stenosis is a rare complication. Pulmonary artery thrombus formation may worsen...
The occurrence of pulmonary artery thrombus in association with rheumatic mitral stenosis is a rare complication. Pulmonary artery thrombus formation may worsen pulmonary artery pressures, and this may precipitate acute right heart failure. The possible mechanisms behind pulmonary artery thrombus formation during mitral valve replacement surgery could be acute coagulopathy following surgery, the presence of chronic pulmonary thromboembolism, or chronic atrial fibrillation. We report an unusual case of pulmonary artery thrombus in a patient with rheumatic MS which was diagnosed with transoesophageal echocardiography after MVR.
Topics: Humans; Heart Valve Prosthesis Implantation; Pulmonary Artery; Echocardiography, Transesophageal; Thrombosis; Mitral Valve; Delayed Diagnosis; Mitral Valve Stenosis; Female; Rheumatic Heart Disease; Pulmonary Embolism; Middle Aged
PubMed: 38722126
DOI: 10.4103/aca.aca_45_23 -
Annals of Cardiac Anaesthesia Jan 2024The quadricuspid aortic valve is a rare congenital anomaly, usually associated with aortic regurgitation requiring surgical intervention. It may be associated with other...
The quadricuspid aortic valve is a rare congenital anomaly, usually associated with aortic regurgitation requiring surgical intervention. It may be associated with other congenital anomalies such as coronary anomalies, patent ductus arteriosus, ventricular septal defect, pulmonary stenosis, and subaortic stenosis. The diagnosis is generally established by either transthoracic or transesophageal echocardiography. Herein, we report a case of a 52-year-old woman who was diagnosed to have quadricuspid aortic valve by intraoperative transesophageal echocardiography.
Topics: Humans; Female; Aortic Valve; Middle Aged; Echocardiography, Transesophageal; Aortic Valve Insufficiency; Heart Defects, Congenital
PubMed: 38722121
DOI: 10.4103/aca.aca_110_23 -
European Heart Journal. Case Reports May 2024Partial anomalous pulmonary venous return (PAPVR) can be surgically corrected using a pericardial baffle. This baffle can become obstructed post-surgery, leading to...
BACKGROUND
Partial anomalous pulmonary venous return (PAPVR) can be surgically corrected using a pericardial baffle. This baffle can become obstructed post-surgery, leading to pulmonary hypertension and right heart dysfunction if not detected and corrected.
CASE SUMMARY
We describe three patients with occluded PAPVR baffles who underwent drug-coated balloon angioplasty and stenting of the obstructed baffle. In each case, baffle obstruction was detected post-operatively on surveillance cross-sectional imaging, and an invasively measured pulmonary capillary wedge-to-left atrium gradient was noted to be elevated. Post-intervention, each patient had an improvement in baffle flow by angiography as well as lung perfusion as assessed by nuclear medicine scintigraphy.
DISCUSSION
Given the subtle symptomatology of obstructed PAPVR pericardial baffle repairs, surveillance imaging is necessary to detect occluded baffles and intervene before downstream right heart disease and pulmonary hypertension develops. Given the high rates of re-stenosis in pulmonary vein stenting, pre-treatment of occluded PAPVR baffles with drug-coated balloons may help reduce re-intervention rates.
PubMed: 38721252
DOI: 10.1093/ehjcr/ytae203 -
Circulation. Cardiovascular Imaging May 2024Quantification of left atrial (LA) conduit function and its contribution to left ventricular (LV) filling is challenging because it requires simultaneous measurements of...
BACKGROUND
Quantification of left atrial (LA) conduit function and its contribution to left ventricular (LV) filling is challenging because it requires simultaneous measurements of both LA and LV volumes. The functional relationship between LA conduit function and the severity of diastolic dysfunction remains controversial. We studied the role of LA conduit function in maintaining LV filling in advanced diastolic dysfunction.
METHODS
We performed volumetric and flow analyses of LA function across the spectrum of LV diastolic dysfunction, derived from a set of consecutive patients undergoing multiphasic cardiac computed tomography scanning (n=489). From LA and LV time-volume curves, we calculated 3 volumetric components: (1) early passive emptying volume; (2) late active (booster) volume; and (3) conduit volume. Results were prospectively validated on a group of patients with severe aortic stenosis (n=110).
RESULTS
The early passive filling progressively decreased with worsening diastolic function (<0.001). The atrial booster contribution to stroke volume modestly increases with impaired relaxation (=0.021) and declines with more advanced diastolic function (<0.001), thus failing to compensate for the reduction in early filling. The conduit volume increased progressively (<0.001), accounting for 75% of stroke volume (interquartile range, 63-81%) with a restrictive filling pattern, compensating for the reduction in both early and booster functions. Similar results were obtained in patients with severe aortic stenosis. The pulmonary artery systolic pressure increased in a near-linear fashion when the conduit contribution to stroke volume increased above 60%. Maximal conduit flow rate strongly correlated with mitral E-wave velocity (r=0.71; <0.0001), indicating that the increase in mitral E wave in diastolic dysfunction represents the increased conduit flow.
CONCLUSIONS
An increase in conduit volume contribution to stroke volume represents a compensatory mechanism to maintain LV filling in advanced diastolic dysfunction. The increase in conduit volume despite increasing LV diastolic pressures is accomplished by an increase in pulmonary venous pressure.
Topics: Humans; Male; Female; Atrial Function, Left; Aged; Diastole; Ventricular Dysfunction, Left; Ventricular Function, Left; Aortic Valve Stenosis; Prospective Studies; Stroke Volume; Severity of Illness Index; Heart Atria; Middle Aged; Aged, 80 and over; Predictive Value of Tests
PubMed: 38716653
DOI: 10.1161/CIRCIMAGING.123.016276 -
European Journal of Case Reports in... 2024Myxoma of the left atrium is a less typical cause of mitral obstruction. If this develops, a flash pulmonary oedema can be the first manifestation.
INTRODUCTION
Myxoma of the left atrium is a less typical cause of mitral obstruction. If this develops, a flash pulmonary oedema can be the first manifestation.
CASE DESCRIPTION
We present a case report of a 50-year-old woman who was admitted to our internal department because of dyspnoea. The patient overcame a stroke three years before the index hospitalisation with a negative transthoracic echocardiography. By anamnesis and physical examination, an exacerbation of COPD was assumed, and the patient was treated accordingly. As the patient showed numerous risk factors for heart failure with preserved ejection fraction, transthoracic echocardiography was performed. A large polypoid mass was found in the left atrium, which caused severe mitral obstruction. Subsequent transoesophageal echocardiography confirmed this finding. The patient underwent urgent cardiac surgery, and the tumour was successfully resected. A histological examination revealed a cardiac myxoma. After the cardiac surgery the patient felt well, and no recurrence of the tumour occurred.
CONCLUSIONS
We provide a case report of a fast-growing myxoma that was incidentally found in a patient with dyspnoea. We highlight the fast growth rate of the tumour and the potential for misdiagnosed signs of pulmonary oedema caused by mitral obstruction.
LEARNING POINTS
Myxomas are the most common primary tumours of the heart, which can manifest a variety of symptoms such as fever, weight loss, thromboembolism, or mitral obstruction.The symptoms of acute exacerbation of COPD and cardiogenic pulmonary oedema can overlap and can be difficult to differentiate by anamnesis and physical examination alone.Transthoracic echocardiography has a high sensitivity for cardiac masses and is the examination of choice when these are suspected.
PubMed: 38715873
DOI: 10.12890/2024_004490