Tricuspid regurgitation associated with cardiac implantable electronic devices (CIED) constitutes a significant subset of secondary tricuspid regurgitation, characterized by a multifactorial etiology involving pacing lead-mediated mechanical interference and CIED-related systemic factors. The pathogenesis of CIED-related tricuspid regurgitation encompasses direct mechanical trauma or functional disruption of the tricuspid valve apparatus by pacing leads, pacing mode-induced hemodynamic alterations, and clinical risk factors such as permanent atrial fibrillation, apical pacing, and high right ventricular pacing burden. The natural progression and clinical outcomes of CIED-related tricuspid regurgitation parallel those of tricuspid regurgitation stemming from other etiologies. Advanced imaging modalities, including echocardiography, cardiac computed tomography, and cardiac magnetic resonance imaging, enable precise diagnosis and longitudinal assessment of CIED-related tricuspid regurgitation. Management strategies emphasize multidisciplinary collaboration as well as integration of preventive approaches-such as refined lead implantation techniques and tailored pacing modalities-with therapeutic interventions ranging from pharmacotherapy to surgical valve repair or replacement. This article reviews the current understanding of CIED-related tricuspid regurgitation to provide a reference for clinical practice and research.
Tricuspid Valve Insufficiency/diagnosis* ; Humans ; Defibrillators, Implantable/adverse effects* ; Pacemaker, Artificial/adverse effects*

Neonatal Marfan syndrome (nMFS) is considered to be on the most severe end of the spectrum of type I fibrillinopathies. The common features of nMFS include ascending aortic dilatation, severe mitral and/or tricuspid valve insufficiency, ectopia lentis, arachnodactyly, joint contractures, crumpled ear, loose skin, and pulmonary emphysema.We describe a newborn male diagnosed with nMFS. He presented several atypical features, such as diaphragmatic eventration, severe hydronephrosis with hydroureter, and dilated cisterna magna. Molecular analysis revealed a missense mutation at nucleotide 3217 (c.3217G>A) in exon 26 of the fibrillin-1 (FBN1) gene, resulting in the substitution of a glutamate for a lysine at codon 1073 (E1073K) in the 12th calcium binding epidermal growth factor-like domain of the FBN1 protein. Here we report a rare case of Nmfs with several combined atypical features, such as diaphragmatic eventration, severe hydronephrosis with hydroureter, and dilated cisterna magna. Our report is the first atypical nMFS case with p.Glu1073Lys mutation of FBN1 in Korea and may help clinicians with the diagnosis and follow-up of atypical nMFS.
Arachnodactyly ; Calcium ; Cisterna Magna ; Codon ; Contracture ; Diagnosis ; Diaphragmatic Eventration ; Dilatation ; Ear ; Ectopia Lentis ; Exons ; Follow-Up Studies ; Glutamic Acid ; Humans ; Hydronephrosis ; Infant, Newborn ; Joints ; Korea* ; Lysine ; Male ; Marfan Syndrome* ; Mutation, Missense ; Skin ; Tricuspid Valve Insufficiency

BACKGROUND/AIMS: Pulmonary endarterectomy (PEA) is the gold standard for treating chronic thromboembolic pulmonary hypertension (CTEPH) in Western countries. The aim of this study was to investigate the long-term outcomes of performing PEA on CTEPH patients in comparison with medical therapy at a single Korean center. METHODS: This retrospective study included 88 CTEPH patients. These patients were classified into the PEA group (n = 37) or non-PEA group (i.e., medical therapy; n = 51). The clinical characteristics, hemodynamic data, and long-term survival rates were compared. Independent prognostic factors for CTEPH were also investigated. RESULTS: CTEPH was not associated with either gender, and the mean age at diagnosis was 53.3 ± 13.7 years. Echocardiography revealed that the mean peak velocity of the tricuspid regurgitation jet was 4.2 ± 0.7 m/sec and the mean pulmonary arterial pressure was 51.7 ± 15.1 mmHg. The PEA and non-PEA groups demonstrated no significant differences, except in terms of the right ventricular end-diastolic diameter. The survival rates of the PEA group were significantly higher than the non-PEA group at 1, 3, 5, and 10 years (p = 0.032). Multivariate analyses indicated that World Health Organization class IV and PEA were significant predictors of poorer and better outcomes, respectively. CONCLUSIONS: PEA demonstrates more favorable effects on long-term survival than medical therapy in Korean CTEPH patients who were considered operable.
Arterial Pressure ; Diagnosis ; Echocardiography ; Endarterectomy ; Hemodynamics ; Humans ; Hypertension, Pulmonary* ; Korea ; Multivariate Analysis ; Peas ; Pulmonary Embolism ; Retrospective Studies ; Survival Rate ; Tricuspid Valve Insufficiency ; World Health Organization

BACKGROUND: Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality among patients with systemic sclerosis (SSc). Early detection and prompt treatment of PAH associated with SSc (SSc-PAH) result in better prognosis. We conducted echocardiographic study to presume the prevalence of PAH in Korean adult SSc patients and to diagnose SSc-PAH in their early stages with right heart catheterization (RHC). METHODS: We performed free of charge echocardiographic study including 37 adult SSc patients at the Chungnam National University Hospital. The possibility of PAH is determined by the estimation of pulmonary arterial pressure by peak tricuspid regurgitation velocity of > 3.0 m/s. Patients with possible PAH were recommended to undergo RHC to confirm the diagnosis. RESULTS: In 37 patients, 8 patients were suspected with PAH. Among them, 6 patients agreed to be examined with RHC, and 4 were confirmed with PAH. The prevalence of possible PAH was 21.6% (8 of 37 patients), and that of confirmed PAH was 10.8% (4 of 37 patients). Four patients who were confirmed with SSc-PAH through RHC have been treated with specific pulmonary vasodilators and maintained stable. CONCLUSION: Eight patients (21.6%) were possible PAH and 4 (10.8%) were diagnosed as SSc-PAH by RHC after the echocardiographic screening study of 37 adult SSc patients.
Adult* ; Arterial Pressure ; Cardiac Catheterization ; Cardiac Catheters ; Chungcheongnam-do ; Diagnosis ; Echocardiography* ; Humans ; Hypertension* ; Mass Screening* ; Mortality ; Prevalence* ; Prognosis ; Scleroderma, Systemic* ; Tricuspid Valve Insufficiency ; Vasodilator Agents

BACKGROUND: Bronchopulmonary dysplasia (BPD) may result in chronic pulmonary artery hypertension and right ventricular (RV) dysfunction. Various echocardiographic assessments of RV dysfunction have been used to determine whether echocardiographic measurements of premature infants with BPD could provide sensitive measures of RV function that correlates with BPD severity. METHODS: Twenty-eight control subjects without BPD (non BPD group), 28 patients with mild BPD, 11 patients with moderate BPD, and six patients with severe BPD underwent echocardiograms with standard measurement such as ejection fraction by M-mode, tricuspid regurgitation pressure gradient, myocardial performance index (MPI) derived from pulse Doppler, and tissue Doppler imaging (TDI) measurements. BPD severity was classified by the NICHD/NHLBI/ORD workshop rating scale. Twenty-eight control subjects without BPD (non BPD group), 28 patients with mild BPD, 11 patients with moderate BPD, and six patients with severe BPD underwent echocardiograms with standard measurement such as ejection fraction by M-mode, tricuspid regurgitation pressure gradient, myocardial performance index (MPI) derived from pulse Doppler, and TDI measurements. BPD severity was classified by the NICHD/NHLBI/ORD workshop rating scale. RESULTS: None of the standard echocardiographic findings was significantly different between the control group and BPD groups. However, mean septal TDI-MPI of the severe BPD group (0.68 ± 0.06) was significantly (p < 0.01) higher than that of the non-BPD (0.58 ± 0.10) or the mild BPD group (0.59 ± 0.12). In addition, mean RV TDI-MPI of the severe BPD group (0.71 ± 0.13) was significantly (p < 0.05) higher than that of the non-BPD group (0.56 ± 0.08) or the mild BPD group (0.60 ± 0.125). Linear regression showed a good correlation between the severity of BPD and RV TDI-MPI (p = 0.01, R = 0.30) or septal TDI-MPI (p = 0.04, R = 0.24). CONCLUSION: Echocardiographic evaluation of RV function based on an assessment of RV TDI-MPI can provide RV dysfunction parameter in premature infants with BPD.
Bronchopulmonary Dysplasia ; Child* ; Diagnosis* ; Echocardiography* ; Education ; Humans ; Hypertension ; Infant, Newborn ; Infant, Premature ; Linear Models ; Prognosis* ; Pulmonary Artery ; Tricuspid Valve Insufficiency ; Ventricular Function, Right

Right ventricular (RV) failure is a complex clinical syndrome characterized by impaired RV filling and ejection. RV function is altered in the setting of either pressure overload or volume overload. RV failure may result from a primary reduction of myocardial contractility caused by ischemia, cardiomyopathy, or arrhythmia, but left ventricular (LV) failure remains the leading cause. As RV dysfunction progresses to RV failure, the RV chamber becomes more spherical and tricuspid regurgitation is aggravated, leading to increasing venous congestion. Ventricular interdependence may result in impaired LV filling, a decrease in LV stroke volume, and low cardiac output. The important clinical manifestations of RV failure are fluid retention, decreased systolic reserve, low cardiac output, or arrhythmias. The initial diagnosis is based on clinical history and physical examination. A chest X-ray, electrocardiogram, and biochemical tests should be routinely obtained to evaluate the underlying causes and comorbidities. Bedside echocardiography provides valuable information on cardiac structure and function. RV ejection fraction is the most commonly used index of RV function although it is a highly load-dependent index.
Arrhythmias, Cardiac ; Cardiac Output, Low ; Cardiomyopathies ; Comorbidity ; Diagnosis* ; Echocardiography ; Electrocardiography ; Heart Failure ; Heart Ventricles ; Hyperemia ; Ischemia ; Physical Examination ; Stroke Volume ; Thorax ; Tricuspid Valve Insufficiency

PURPOSE: Infantile Marfan syndrome (MFS) is a rare congenital inheritable connective tissue disorder with poor prognosis. This study aimed to evaluate the cardiovascular manifestations and overall prognosis of infantile MFS diagnosed in a tertiary referral center in Korea. METHODS: Eight patients diagnosed with infantile MFS between 2004 and 2014 were retrospectively evaluated. RESULTS: Their median age at the time of diagnosis was 2.5 months (range, 0-20 months). The median follow-up period was 25.5 months (range, 0-94 months). The median length at birth was 50.0 cm (range, 48-53 cm); however, height became more prominent over time, and the patients were taller than the 97th percentile at the time of the study. None of the patients had any relevant family history. Four of the 5 patients who underwent DNA sequencing had a fibrillin 1 gene mutation. All the patients with echocardiographic data of the aortic root had a z score of >2. All had mitral and tricuspid valve prolapse, and various degrees of mitral and tricuspid regurgitation. Five patients underwent open-heart surgery, including mitral valve replacement, of whom two required multiple operations. The median age at mitral valve replacement was 28.5 months (range, 5-69 months). Seven patients showed congestive heart failure before surgery or during follow-up, and required multiple anti-heart failure medications. Four patients died of heart failure at a median age of 12 months. CONCLUSION: The prognosis of infantile MFS is poor; thus, early diagnosis and timely cautious treatment are essential to prevent further morbidity and mortality.
Connective Tissue ; Diagnosis ; Early Diagnosis ; Echocardiography ; Follow-Up Studies ; Heart Failure ; Humans ; Infant, Newborn ; Korea ; Marfan Syndrome* ; Mitral Valve ; Mitral Valve Insufficiency ; Mortality ; Parturition ; Prognosis ; Retrospective Studies ; Sequence Analysis, DNA ; Tertiary Care Centers* ; Tricuspid Valve Insufficiency ; Tricuspid Valve Prolapse

Current knowledge of functional tricuspid regurgitation (FTR) as a progressive entity, worsening the prognosis of patients irrespective of its aetiology, has led to renewed interest in the pathophysiology and assessment of FTR. For the proper management of FTR, not only its severity, but also the mechanisms, the mode of leaflet coaptation, the degree of tricuspid annulus enlargement and leaflet tenting, and the haemodynamic consequences for right atrial and right ventricular morphology and function have to be taken into account. A better assessment of the anatomy and function of tricuspid apparatus and tricuspid regurgitation severity should help with the appropriate selection of patients who will benefit from either surgical tricuspid valve repair/replacement or a percutaneous procedure, especially among patients who are to undergo or have undergone primary left-sided valvular surgery. In this article, we review the anatomy, pathophysiology and the use of imaging techniques to assess patients with FTR, as well as the various treatment options for FTR, including emerging transcatheter procedures. The limitations affecting the current approach to FTR patients and the unmet clinical needs for their management have also been discussed.
Diagnosis* ; Echocardiography ; Humans ; Prognosis ; Tricuspid Valve ; Tricuspid Valve Insufficiency*

Current knowledge of functional tricuspid regurgitation (FTR) as a progressive entity, worsening the prognosis of patients irrespective of its aetiology, has led to renewed interest in the pathophysiology and assessment of FTR. For the proper management of FTR, not only its severity, but also the mechanisms, the mode of leaflet coaptation, the degree of tricuspid annulus enlargement and leaflet tenting, and the haemodynamic consequences for right atrial and right ventricular morphology and function have to be taken into account. A better assessment of the anatomy and function of tricuspid apparatus and tricuspid regurgitation severity should help with the appropriate selection of patients who will benefit from either surgical tricuspid valve repair/replacement or a percutaneous procedure, especially among patients who are to undergo or have undergone primary left-sided valvular surgery. In this article, we review the anatomy, pathophysiology and the use of imaging techniques to assess patients with FTR, as well as the various treatment options for FTR, including emerging transcatheter procedures. The limitations affecting the current approach to FTR patients and the unmet clinical needs for their management have also been discussed.
Diagnosis* ; Echocardiography ; Humans ; Prognosis ; Tricuspid Valve ; Tricuspid Valve Insufficiency*

Unguarded tricuspid regurgitation (TR) due to a flail tricuspid leaflet is a rare condition of newborn cyanosis. A high perinatal mortality has been associated with this fatal condition. But, there are feasible surgical repairs to improve survival. We report the case of a male full-term neonate with intractable hypoxia. He had profound tricuspid insufficiency and leaflet prolapse caused by a ruptured papillary muscle supporting the anterior leaflet of the tricuspid valve. He presented with severe cyanosis and respiratory distress immediately after birth. Despite medical management, the pulmonary vascular resistance was not decreased and a low cardiac output persisted. Initial stabilization was accomplished with nitric oxide and extracorporeal membrane oxygenation. The tricuspid valve repair surgery was successfully performed subsequently. TR resulting from papillary muscle rupture is a potentially lethal condition. Timely diagnosis and proper surgical treatment can be lifesaving.
Anoxia ; Cardiac Output, Low ; Cyanosis ; Diagnosis ; Extracorporeal Membrane Oxygenation ; Female ; Humans ; Infant, Newborn* ; Male ; Nitric Oxide ; Papillary Muscles* ; Parturition ; Perinatal Mortality ; Persistent Fetal Circulation Syndrome* ; Prolapse ; Rupture ; Thoracic Surgery ; Tricuspid Valve Insufficiency ; Tricuspid Valve* ; Vascular Resistance