Implante valvular percutáneo valve-in-valve en tricúspide. Primer reporte de caso de paciente pediátrico en Colombia / Percutaneous valve-in-valve valve implantation in tricuspid position. First case report of a pediatric patient in Colombia

Resumen Las anormalidades de la válvula tricúspide tienen una incidencia menor respecto a los defectos del resto de válvulas cardiacas y pueden deberse a causas primarias o secundarias. Recientemente, se ha estudiado su fisiopatología y su importancia en la morbimortalidad por causas cardiacas. En este sentido, la cirugía valvular tricúspide abierta tiene una tasa de morbimortalidad elevada, más aún cuando se trata de una reoperación. Es por esto que estos procedimientos endovasculares transcatéter cobran relevancia y recientemente se han publicado a lo largo del mundo algunos casos de intervenciones por este método, por medio de implante valvular percutáneo valve-in-valve en posición tricúspide. Se presenta el primer caso reportado de este procedimiento en Colombia en una paciente pediátrica, el cual transcurrió sin complicaciones y con el que se logró una mejoría significativa en la función valvular como paso inicial para continuar desarrollando la técnica y realizando este procedimiento en el país.

Abstract Abnormalities in the tricuspid valve have a lower incidence than defects in the rest of the heart valves and may be due to primary or secondary causes. Recently, its pathophysiology and importance in morbidity and mortality due to cardiac causes have been widely studied. Open tricuspid valve surgery has a high rate of morbidity and mortality, and even more so when it comes to reoperation. For this reason, it is important to carry out these procedures endovascularly via a transcatheter catheter and some cases of exacerbations by this method have recently been published throughout the world, by means of percutaneous valve-in-valve implantation in the tricuspid position. We present the first reported case of this procedure in Colombia in a pediatric patient, whose procedure was uneventful and a significant improvement in valve function was impaired, as an initial step to continue developing the technique and performing the procedure in this country.

Observation of the prophylactic use of prokinetic agents in patients after tricuspid valve replacement under cardiopulmonary bypass / 药学实践与服务

Objective To analyze the postoperative therapeutic effect of prokinetic agents in patients after tricuspid valve replacement （TVR） under cardiopulmonary bypass. Methods Patients received TVR under cardiopulmonary bypass （during June 2010 to December 2021） in the department of Cardiovascular Surgery of the First Affiliated Hospital of Naval Medical University were selected as the subjects of our study. The data of basic characteristics, first postoperative defecation time and postoperative recovery condition were collected and retrospectively analyzed in the patients taking prokinetic agents within three days after surgery （prevention group） and patients not taking prokinetic agents within three days after surgery （control group）. Results A total of 184 patients were selected, including 101 in the prevention group and 83 in the control group. The first defecation time of patients in the prevention group was significantly earlier than that in the control group （P<0.05）. The incidences of abdominal distension and pulmonary infection in the prevention group were significantly lower than that in the control group. The length of time in ICU, postoperative mechanical ventilation and nasogastric tube decompression in the prevention group were significantly shorter than that in the control group （P<0.05）. Conclusion Taking prokinetic agents within three days after TVR under cardiopulmonary bypass could effectively improve the prognosis of patients.

Research advances in animal models of tricuspid regurgitation / 复旦学报（医学版）

Tricuspid regurgitation(TR)cases are widely distributed in China.Poor clinical drug efficacy,high surgical risk,and poor prognosis for right heart failure are found in patients with moderate or severe TR.In recent years,with the innovation of valve instruments and the development of technology,transcatheter tricuspid valve treatment could be a new choice for high-risk TR patients in surgery.Many TR animal models have emerged these years for the research of the mechanism of TR and for the clinical verification of instruments.Therefore,this review focuses on how to develop an animal model of TR and discusses the advantages and disadvantages of these techniques.

Additional tricuspid valve annuloplasty versus isolated closure for atrial septal defect with secondary moderate to severe tricuspid regurgitation: A propensity score matching study / 中国胸心血管外科临床杂志

@#Objective To compare the efficacy of additional tricuspid valve annuloplasty (TVP) and isolated closure for atrial septal defect (ASD) with moderate to severe tricuspid regurgitation (TR). Methods Clinical data of the patients diagnosed with ASD combined with secondary moderate to severe TR and treated in our hospital from January 2009 to June 2020 were retrospectively analyzed. Patients were divided into a TVP group and a non-TVP group based on whether TVP was performed simultaneously. The baseline data of two groups were matched with a ratio of 1∶1 propensity score. Results A total of 32 pairs from 257 patients were successfully matched. In the TVP group, there were 24 females and 8 males with an average age of 44.0±13.1 years. In the non-TVP group, there were 28 females and 4 males with an average age of 44.5±11.6 years. The TR area and estimated pulmonary artery pressure in the two groups were significantly decreased compared with preoperation (all P<0.001). The TR area (P=0.001) and the estimated pulmonary artery pressure (P=0.002) were decreased more significantly in the TVP group than those in the non-TVP group. Linear regression analysis showed that age and preoperative TR area had a positive correlation with TR area at follow-up (β=0.045 and 0.259, respectively, both P<0.05), while additional TVP had a negative correlation (β=–1.542, P=0.001). Conclusion Additional TVP can significantly reduce the TR area and pulmonary artery pressure, and elderly patients with severe TR before surgery should actively receive TVP.

A Successful Case of Redo-Tricuspid Valve Replacement for Prosthetic Valve Endocarditis Thirty-Two Years after Single Tricuspid Bioprosthetic Valve Replacement / 日本心臓血管外科学会雑誌

We present a successful case of redo-tricuspid valve replacement for tricuspid prosthetic valve endocarditis. A 78-year-old man who underwent tricuspid bioprosthetic valve replacement for severe tricuspid regurgitation thirty-two years earlier was referred to our institution with persistent high fever and back pain. The blood culture was positive for Streptococcus oralis, and echocardiography revealed a mobile vegetation attached to the tricuspid prosthetic valve with moderate tricuspid regurgitation. A clinical diagnosis of prosthetic valve endocarditis was established. Redo-tricuspid bioprosthetic valve replacement was performed following antibiotics therapy. The patient was discharged on postoperative day 49 after 6 weeks of additional antibiotic treatment, and had no recurrence of infection for 6 months after redo-surgery.

A Case of Infective Endocarditis Treated with Ventricular Septal Defect Closure with the Sandwich Method, Aortic Valve Replacement, and Tricuspid Valve Replacement / 日本心臓血管外科学会雑誌

A 74-year-old man was diagnosed with infective endocarditis (IE) involving the aortic and tricuspid valves, ventricular septal defect (VSD), and complete atrioventricular block. He was admitted to a previous hospital with complaints of fever and neck pain, and he developed complete atrioventricular block during the course of his illness. An echocardiogram revealed severe aortic regurgitation, aortic valve vegetations, and a ventricular septal defect. He was then transferred to our hospital, and he underwent emergent surgery. The aortic valve cusps were calcified and thick, with significant cusp destruction. The vegetations partly extended to the subvalvular area of the right and non-coronary cusp. The vegetations also extended from the atrial septum to the tricuspid valve septal leaflet and perimembranous VSD. Ventricular septal reconstruction using the sandwich technique with two bovine pericardial patches, aortic valve replacement, and tricuspid valve replacement were performed. Postoperatively, he received antibiotic therapy for six weeks and was discharged from our hospital after the implantation of a cardiac resynchronization therapy pacemaker. Echocardiography showed no residual shunts. Our case suggests that the sandwich technique can be a useful method of septal reconstruction for IE with extensive destruction of the ventricular septum.

Current applications of transcatheter edge-to-edge tricuspid valve repair / 中国介入心脏病学杂志

Tricuspid regurgitation(TR)is a common heart valve disease.According to the pathogenesis,TR can be divided into primary(organic)and secondary(functional)regurgitation,of which functional TR accounts for more than 90%.Patients with severe TR have poor prognosis and poor drug treatment,and surgery(valvuloplasty)is the main treatment.At present,transcatheter edge-to-edge tricuspid valve repair(T-TEER)has become an essential program of transcatheter treatment for TR,providing minimally invasive treatment for TR patients who cannot undergo surgery or are at high risk of surgery.T-TEER reduces the degree of regurgitation by clamping leaflets,and is currently in the early stage of research and development exploration and clinical validation,mainly for functional TR.T-TEER devices have also made significant progress(TriClip,PASCAL),and Chinese-made novel-designed T-TEER devices are also undergoing clinical trials(DragonFly-TTM,SQ-Kyrin-TTM,NeoBlazarTM).This paper reviews the current applications and research progress of T-TEER.

Tricuspid Valve Geometry of Idiopathic Pulmonary Hypertension:a Three-dimensional Transthoracic Echocardiography Study / 中国循环杂志

Objectives:To evaluate the tricuspid valve(TV)geometric remodeling in patients with idiopathic pulmonary arterial hypertension(IPAH)by three-dimensional transthoracic echocardiography. Methods:Two-dimensional and three-dimensional transthoracic echocardiography were performed in 30 IPAH patients and 15 healthy controls,and the geometry parameters of TV were obtained by four-dimensional auto tricuspid valve quantitative(4D Auto-TVQ)in the right ventricular-focused apical view.Pulmonary arterial hypertension was determined by right heart catheterization within 48 hours of echocardiography. Results:The 4-chamber diameter,tricuspid annular(TA)perimeter,TA area,maximal tenting height,coaptation point height and tenting volume were larger in IPAH patients than those in healthy controls(all P＜0.05),2-chamber diameter was similar between two groups.In IPAH group,maximal tenting height and coaptation point height were moderately correlated with right ventricular end-diastolic volume(r=0.710,r=0.515,both P＜0.05),while TA perimeter,4-chamber diameter and TA area were moderately correlated with right atrial end-systolic volume(r=0.712,r=0.558,r=0.545,all P＜0.05). Conclusions:IPAH patients have larger maximal tenting height,coaptation point height and tenting volume,TA enlargement is mainly visible in 4-chamber diameter.TV tenting height is associated with right ventricular volume,but TA size is associated with right atrial volume in IPAH patients.

Cirugia de la insuficiencia tricuspidea / Tricuspid regurgitation surgery

La valvulopatía tricuspídea es considerada frecuente y sobre todo en pacientes con afectación mitral, habiendo sido sostenidamente objeto de controversias, tanto en la selección de la correcta actuación terapéutica, como del momento apropiado de su ejecución. Aunque la válvula tricúspide es afectada por estenosis, predominantemente es asiento de lesión tipo insuficiencia, que significa paso retrógrado de sangre desde el ventrículo derecho (VD) a la aurícula derecha durante la sístole. Puede ser primaria (orgánica) o secundaria (funcional), relacionada a dilatación/disfunción ventricular derecha, dilatación anular, tracción de sus valvas e hipertensión arterial pulmonar. La ecocardiografía tridimensional (3D) y la resonancia magnética cardiaca al permitir cuantificar con mayor precisión el tamaño del VD y la función sistólica, han permitido actuaciones terapéuticas más tempranas y con mejores resultados, que incluyen la reparación valvular tricuspídea, sustitución valvular y diversas modalidades de técnicas transcatéter. Con estas consideraciones, presentamos a consideración de ustedes el estado actual de la cirugía en la insuficiencia tricuspídea.

Tricuspid valve disease is considered common and especially in patients with mitral involvement, having been the subject of sustained controversy, both in the selection of the correct therapeutic action and the appropriate moment of its execution. Although the tricuspid valve is affected by stenosis, it is predominantly the seat of an insufficiency-type lesion, which means retrograde passage of blood from the right ventricle (RV) to the right atrium during systole. It can be primary (organic) or secondary (functional), related to right ventricular dilation/dysfunction, annular dilation, traction of its leaflets and pulmonary arterial hypertension. Three- dimensional (3D) echocardiography and cardiac magnetic resonance, by allowing more precise quantification of RV size and systolic function, have allowed earlier therapeutic actions with better results, which include tricuspid valve repair, valve replacement and various modalities of transcatheter techniques. With these considerations, we present for your consideration the current state of surgery in tricuspid regurgitation.

Estenosis tricuspídea de bioprótesis tratada mediante implante valvular percutáneo: una solución segura para un paciente inusual / Percutaneous Resolution for Tricuspid Stenosis: Positive Outcomes Despite High Surgical Risk

Se comunica el caso de una mujer de 31 años quien había sido previamente sometida a reemplazo valvular aórtico, mitral y tricuspídeo en tres episodios quirúrgicos. Fue ingresada a nuestra sección de cardiologÍa con síntomas compatibles con insuficiencia cardiaca derecha. Se efectuó evaluación mediante ecocardiograma, sondeo cardíaco derecho/izquierdo, angioTAC cardíaco y cardio resonancia. Se diagnosticó una estenosis tricuspídea severa secundaria a disfunción de prótesis biológica. Debido a alto riesgo operatorio y riesgo de falla ventricular derecha post-operatorio, la paciente fue rechazada para reemplazo valvular quirúrgico. Se decidió efectuar reemplazo tricuspídeo procedimiento "valve in valve". Se logró efectuar de manera exitosa por abordeje venoso femoral, prótesis balón expansible. Se demostró corrección de la estenosis tricuspídea y la paciente evolucionó con remisión de los síntomas de falla cardíaca.

We report a 31-year-old woman who was previously subjected to aortic, mitral, and tricuspid valve replacement in three different surgical episodes. She was admitted to our cardiology section with acute right heart failure symptoms. She was studied by echocardiography, right/left heart catheterization, cardiac CT scan and cardiac magnetic resonance. A severe tricuspid stenosis due to biological prosthesis dysfunction was diagnosed. Due to high operative risk and risk of postoperative right ventricular failure, the patient was rejected for cardiovascular surgery. We decided on a percutaneous tricuspid "valve in valve replacement". The procedure was done successfully by venous femoral approach, with a balloon expandable prosthesis. Tricuspid stenosis was corrected and the patient evolved with remission of heart failure symptoms.

Como eu faço a avaliação pós-intervenções tricúspides: dicas e truques / My approach to assessment after tricuspid interventions: tips and tricks

A regurgitação tricúspide (RT) importante está associada à alta morbidade e mortalidade. Como o tratamento cirúrgico da RT isolada tem sido associado à alta mortalidade, as intervenções transcateter na valva tricúspide (VT) têm sido utilizadas para o seu tratamento, com risco relativamente mais baixo. Há um atraso na intervenção da RT e provavelmente está relacionado a uma compreensão limitada da anatomia da VT e do ventrículo direito, além da subestimação da gravidade da RT. Nesse cenário, faz-se necessário o conhecimento anatômico abrangente da VT, a fisiopatologia envolvida no mecanismo de regurgitação, assim como a sua graduação mais precisa. A VT tem peculiaridades anatômica, histológica e espacial que fazem a sua avalição ser mais complexa, quando comparado à valva mitral, sendo necessário o conhecimento e treinamento nas diversas técnicas ecocardiográficas que serão utilizadas frequentemente em combinação para uma avaliação precisa. Esta revisão descreverá a anatomia da VT, o papel do ecocardiograma no diagnóstico, graduação e fisiopatologia envolvida na RT, as principais opções atuais de tratamento transcateter da RT e a avaliação do resultado após intervenção transcateter por meio de múltiplas modalidades ecocardiográficas.(AU)

Severe tricuspid regurgitation (TR) is associated with high morbidity and mortality. Given that surgical treatment of TR alone has been associated with high mortality, transcatheter interventions in the tricuspid valve (TV) have been used for its treatment, with relatively lower risk. There is a delay in intervention for TR, and this is probably related to a limited understanding of the anatomy of the TV and the right ventricle, in addition to an underestimation of the severity of TR. In this scenario, it is necessary to have comprehensive anatomical knowledge of the TV, the pathophysiology involved in the mechanism of regurgitation, and more accurate grading. The TV has anatomical, histological, and spatial peculiarities that make its assessment more complex when compared to the mitral valve, requiring knowledge and training in the various echocardiographic techniques that will often be used in combination for accurate assessment. This review will describe the anatomy of the TV, the role of echocardiography in the diagnosis, grading, and pathophysiology involved in TR; the main transcatheter treatment options currently available for TR; and the assessment of outcomes after transcatheter intervention by means of multiple echocardiographic modalities.(AU)

Comparative Morphological and Morphometrical Analysis of Atrio-Ventricular Valves of Human and Porcine

There is increased incidence of valvular heart diseases in recent years due to life style modifications. The mortality rates in valvular diseases are kept in pace using various modalities of treatments. One such lifesaving treatment is valve replacement surgeries. These are done by using mechanical valve prosthesis or tissue grafts. The tissue valves prosthesis, harvested from porcine heart are called as xenograft and are increasingly used in valve repair and replacement surgeries. In the present scenario, there is a smaller number of systematically analysed literatures available on the comparative anatomy of human and porcine heart valves. Hence this study was carried out to acquire knowledge and to put forth some points to future research works on heart valves. In this study, 20 formalin fixed porcine and human hearts were procured from slaughter house and cadavers respectively. The morphology and morphometry of tricuspid valve and mitral valve was observed and analysed using spss software 20 version. All the dependent variables were compared using student t test and independent sample test. The results were tabulated and compared. It was observed that the tricuspid and the mitral valve of the porcine resembles the corresponding human heart valves in morphology and morphometry and their values were coinciding to their maximum. The porcine valve resembles human heart valves in morphology and it can be used in designing valve substitutes in replacement surgeries. Porcine valve can also be used as bio-prosthesis by matching the morphometry and by reducing the geometrical difference to their minimum by using any interventional radiology.

Tricuspid Valve-in-Valve Procedure with An Edwards S3 Valve in a 15-kg Child in Latin America

ABSTRACT A 5-year-old child, weighing 15 kg, with three previous sternotomies, presented with right heart failure due to severe stenosis and regurgitation of the bioprosthetic tricuspid valve. A percutaneous tricuspid valve-in-valve procedure with an Edwards S3 valve was ofered for compassionate use, performed with no complications and with a significant clinical condition improvement.

Total Thoracoscopic Surgery for Late Mitral Paravalvular Leakage Repair in A Beating Heart

Abstract Paravalvular leakage (PVL) after mitral valve replacement is a troublesome complication that may lead to severe symptoms and reoperation. Previous case reports on total thoracoscopic cardiac surgery without aortic cross-clamping for repairing late PVL are rare. We describe a 64-year-old man who had undergone aortic and mitral valve replacement via median sternotomy eight years earlier, and who recently developed cardiac failure due to severe tricuspid regurgitation (TR) and PVL in the posterior mitral annulus. During total thoracoscopic surgery with using the beating heart technique, direct closure of the PVL was achieved via pledgeted mattress sutures, and tricuspid valvuloplasty was routinely performed to treat TR. This case indicated that total thoracoscopic surgery on a beating heart may be an excellent option for treating PVL concomitant with TR.

Tricuspid Annuloplasty and Mitral Valvuloplasty for Tricuspid and Mitral Regurgitation with Severe Right Heart Failure Complicated with Liver Cirrhosis / 日本心臓血管外科学会雑誌

A 71-year-old woman underwent VVI pacemaker implantation for complete atrioventricular block 38 years ago at the cardiovascular department of another hospital. In the course of observation, she developed atrial fibrillation. One year ago, she was admitted to hospital for aggravated congestive heart failure and was subsequently treated by a cardiovascular practitioner. She had persistent shortness of breath and lower leg edema, which were treated with increasing doses of oral diuretics. Due to poor treatment outcomes, she was referred to our hospital. Her echocardiography results suggested severe tricuspid regurgitation (TR) and moderate mitral regurgitation. She was also found to have impaired renal function and liver cirrhosis (Stage A of the Child classification), and was admitted. In addition, she had undernutrition with suspected cardiac cachexia. She was first treated by inotrope infusion and central venous hyperalimentation before tricuspid annuloplasty and mitral valvuloplasty were performed. Even though her postoperative management was complicated, she was discharged from our hospital. The conservative treatment with the increased dose of the diuretic for TR-associated right heart failure was prolonged in this patient, leading to severe right heart failure and aggravation of impaired renal function or congestive liver-associated hepatic disorders. Eventually, the patient required operative intervention; however, postoperative management is usually complex, and the operation result may be poor in such patients. We suggest that, from the time when right heart failure can be managed with relatively small doses of diuretics, surgeons should be involved in the care of patients with severe TR who do not require left heart valvular surgery, and should discuss the necessity of surgery earlier than the relevant guidelines suggest, depending on the patient's condition.

Concomitant repair of moderate or less tricuspid regurgitation during mitral valve surgery improves early and mid-term prognosis of tricuspid valve and right heart function / 中国胸心血管外科临床杂志

@#Objective    To investigate the effect of concomitant tricuspid valve repair during mitral valve surgery on the early and mid-term prognosis of the tricuspid valve and right heart function in the patients with moderate or less tricuspid regurgitation. Methods    A retrospective study of 461 patients with mitral valve disease requiring cardiac surgery combined with moderate or less tricuspid regurgitation in our hospital from 2011 to 2014 was done. They were 309 males and 152 females with a median age of 53.00 (44.00, 60.00) years. According to whether they received tricuspid valve repair (Kay’s annuloplasty, DeVega’s annuloplasty or annular ring implantation), the patients were divided into a mitral valve surgery only group (a nTAP group, n=289) and a concomitant tricuspid valve repair group (a TAP group, n=172). At the same time, 43 patients whose tricuspid valve annulus diameter was less than 40 mm in the TAP group were analyzed in subgroups. Results    The median follow-up duration was 3.00 years (range from 0.10 to 9.30 years). There was no perioperative death. Three months after surgery, the anteroposterior diameter of the right ventricle in the TAP group was significantly improved compared with that in the nTAP group [–1.00 (–3.00, 1.00) mm vs. 0.00 (–0.20, 2.00) mm, P=0.048]. Three years after surgery, the improvement of right ventricular anteroposterior diameter in the TAP group was still significant compared with the nTAP group [–1.00 (–2.75, 2.00) mm vs. 2.00 (–0.75, 4.00) mm, P=0.014], and the patients in the TAP group were less likely to develop moderate or more tricuspid regurgitation (3.64% vs. 35.64%, P<0.001). Annuloplasty ring implantation was more effective in preventing regurgitation progression (P=0.044). For patients with a tricuspid annulus diameter less than 40 mm, concomitant tricuspid valve repair was still effective in improving the anteroposterior diameter of the right ventricle in the early follow-up (P=0.036). Conclusion     Concomitant tricuspid valve repair for patients with moderate or less tricuspid regurgitation during mitral valve surgery can effectively improve the tricuspid valve and right heart function in the early and mid-term after surgery. Annuloplasty ring implantation is more effective in preventing regurgitation progression. Patients whose tricuspid annulus diameter is less than 40 mm can also benefit from concomitant tricuspid repair.

Pharmaceutical care in a case of myocardial infarction complicated with antithrombotic therapy after tricuspid valve repair / 药学实践与服务

Objective To investigate the antithrombotic therapy for acute myocardial infarction patients after PCI stenting combined with tricuspid valve repair. Methods The risk of bleeding and embolization was evaluated, relevant data were reviewed, professional knowledges of pharmacy were utilized through the whole treatment process, individualized medication plan for patients was designed and used. Results A better therapeutic effect was achieved through the implementation of pharmaceutical care and medication education to the patients. Conclusion Clinical pharmacists carry out pharmaceutical care in antithrombotic drugs utilities，which could improve the safety level of drug use and provide basis for clinical rational drug use.

Ultrasonic diagnosis of fetal congenitally unguarded tricuspid valve orifice: analysis of two cases and literature review / 中华围产医学杂志

Objective:To review the imaging characteristics and evaluate the diagnostic value of echocardiography for fetal congenitally unguarded tricuspid valve orifice (CUTVO).Methods:Clinical data and ultrasonic images of two pregnant women diagnosed with fetal CUTVO through ultrasound and confirmed by pathology in Nanchang First Hospital between April 2019 and December 2021 were retrospectively analyzed. Relevant literature on confirmed CUTVO diagnosis cases with ultrasonic diagnosis results was searched in the Wanfang database, CNKI database, VIP database, Chinese Medical Journal database, PubMed, and Web of Science database. Descriptive statistical analysis was used to analyze the data.Results:The study involved 17 patients, 15 of whom were retrieved from the databases and two cases from our hospital. Among the 17 cases, 16 were singleton pregnancies, while one case involved a monochorionic monoamniotic twin pregnancy. The tricuspid valve was completely absent or hypoplastic in ten cases, and partial absence of the tricuspid leaflet was found in seven cases. Nine cases had pulmonary atresia with intact ventricular septum and two with ventricular septal defect, while complications were not described in the other six cases. Among the six misdiagnosed cases, four were prenatally misdiagnosed as fetal Ebstein malformation, two cases for Uhl's disease and suspected noncompaction of the ventricular myocardium. CUVTO were confirmed after pregnancy termination. Both cases of missed diagnosis were in the second trimester with no significant enlargement in the right heart, including one with twin transfusion syndrome and one with pulmonary valve stenosis. Nine cases terminated their pregnancies and seven of them had confirmed CUTVO by autopsy after an induced labor. Eight fetuses were delivered. Except for two deaths due to severe conditions, six neonates survived and underwent surgical treatment due to atrial septal defect and patent ductus arteriosus on postnatal echocardiography examination. Among them, two cases underwent tricuspid valve plasty and the tricuspid valve regurgitated moderately during follow-up within one year after surgery; two underwent tricuspid valve replacement with mechanical valves and postoperative less moderate regurgitation was also found; modified Fontan surgery was conducted in one case and the cardiac function turned normal; in one case, no surgical modality was described. Typical echocardiographic features of CUTVO were as follows: normal atrioventricular structure and the connection to arteries; total or partial absence of tricuspid leaflets and papillary muscles; the annulus of the tricuspid valve can be described as "empty" in the apical four-chamber view; Doppler evaluation shows to-and-fro flow across the tricuspid orifice with low velocity and a two-way spectrum.Conclusion:Typical echocardiography features of CUTVO were absence of tricuspid leaflets and moderate to massive regurgitation with a two-way spectrum. It is essential to make efforts to minimize missed and misdiagnoses through dynamic observation of multiple sections.