Late-gestation prediction of outcome in tricuspid valve dysplasia and Ebstein's anomaly using fetal tricuspid regurgitation waveform analysis.

OBJECTIVE: To investigate the criteria, based on fetal TR waveforms in late gestation, to predict biventricular circulation (BV) after birth in cases of tricuspid valve dysplasia (TVD) or Ebstein's anomaly diagnosed during the fetal period. METHODS: We included 35 consecutive cases diagnosed with TVD or Ebstein's anomaly during the fetal period between January 2008 and December 2021 at Kanagawa Children's Medical Center, Kanagawa, Japan. The maximum velocity and change in pressure over time of tricuspid regurgitation (TR) jet (dP/dt), estimated using TR waveforms obtained during the late-gestation period (gestational age ≥ 28 weeks), were collected from patient records. dP/dt was calculated by dividing the change in estimated right ventricular pressure obtained using Bernoulli's principle by the time taken for the TR maximum velocity to change from one-third to two-thirds of its peak value. The outcome was divided into four categories: BV, single ventricular circulation, neonatal death and fetal death. Patients with BV were included in the BV group, while patients with single ventricular circulation, neonatal death or fetal death were included in the non-BV (NBV) group. RESULTS: Overall, 19 and 16 patients were included in the BV and NBV groups, respectively. The median TR maximum velocity was 3.3 (range, 2.4-3.6) m/s in the BV group and 1.9 (range, 1.0-3.3) m/s in the NBV group. There were no cases of postnatal BV in fetuses with TR maximum velocity < 2.4 m/s; cases with TR maximum velocity of 2.4-3.3 m/s were observed in both BV and NBV groups. Receiver-operating-characteristics-curve analysis was performed on the 11 patients in the BV group and five patients in the NBV group with a TR maximum velocity of 2.4-3.3 m/s. dP/dt ≥ 350 mmHg/s and TR maximum velocity ≥ 2.9 m/s were identified as criteria for predicting the outcome in such cases. The performance of dP/dt ≥ 350 mmHg/s in predicting BV after birth in fetuses with TVD or Ebstein's anomaly was higher compared to that of TR maximum velocity ≥ 2.9 m/s (sensitivity, 90.9% vs 72.3% and specificity, 80.0% vs 80.0%, respectively). CONCLUSIONS: In fetuses with TVD or Ebstein's anomaly, the postnatal outcome may be BV or NBV when the TR maximum velocity is 2.4-3.3 m/s. In such cases, by combining the TR maximum velocity with dP/dt ≥ 350 mmHg/s, BV after birth may be predicted with greater accuracy. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.

Tricuspid valve repair with papillary muscle approximation for congenital tricuspid valve regurgitation due to tricuspid valve dysplasia.

We experienced a case of a 1-year-old female with congenital tricuspid valve regurgitation caused by tricuspid valve dysplasia. The anterior and septal leaflets were particularly dysplastic, and leaflet tethering was observed. The anterior papillary muscle was approximated to the interventricular septum, and a commissural edge-to-edge suture was inserted on the anteroseptal commissure. Tricuspid valve regurgitation improved to be trivial after surgery and has not worsened 1 year later. Papillary muscle approximation could be one of the feasible reparative techniques for congenital tricuspid valve regurgitation.

Outcomes of surgical management of Ebstein anomaly and tricuspid valve dysplasia in critically ill neonates and infants.

Objective: To describe the surgical outcomes in neonates and infants who had surgery for Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD). Methods: Retrospective chart review for all patients who underwent surgery for EA or TVD during the index hospitalization after birth at our institution from January 2005 to February 2023. Results: Fifteen symptomatic neonates and infants who had surgery for EA or TVD were included, 8 with EA and 7 with TVD. Eleven patients (73%) and 3 patients (20%) required preoperative inotropes and extracorporeal membrane oxygenation, respectively. Nine patients (60%) had a Starnes procedure and 6 patients (40%) had tricuspid valve repair (TVr). Mortality at last follow-up was 27% overall (n = 4/15), 22% after Starnes (n = 2/9) and 33% after TVr (n = 2/6), without a significant difference despite a greater-risk profile in the Starnes group. Postoperative day 1 lactate level was associated with mortality on Cox regression (hazard ratio, 1.45; P = .01). Three of 9 patients who had a Starnes procedure were or will be converted to a cone repair (1.5/2-ventricle repair). Conclusions: Mortality after surgery for EA or TVD during the index hospitalization after birth is still significant in the current era and is associated with a greater lactate level at postoperative day 1. The Starnes procedure and TVr had comparable outcomes despite a greater-risk profile in the Starnes group. An initial single-ventricle approach does not preclude conversion to biventricular or 1.5-ventricle repair.

The Association of Pulmonary Flow Characteristics With Cardiac Function in Tricuspid Valve Malformation Fetuses: Study With Two-Dimensional Speckle Tracking Echocardiography.

OBJECTIVES: To explore the relationship between cardiac function and pulmonary flow characteristics in tricuspid valve malformation (TVM) fetuses by conventional and speckle tracking echocardiography. METHODS: Eighty-eight TVM fetuses were retrospectively included and compared with 80 normal controls. TVM fetuses in each trimester were divided into two subgroups according to pulmonary flow characteristics: those with normal pulmonary flow (TVM-N) and those with reduced or absent pulmonary flow (TVM-R/A). Cardiac measurements, Celermajer index, and Simpson-Andrews-Sharland (SAS) score were obtained. Speckle tracking echocardiography was used to compute ventricular deformation parameters. RESULTS: TVM fetuses demonstrated significantly lower global longitudinal strain (GLS) and strain rate for both ventricles than controls (all P < .05). When compared with TVM-N, left ventricular (LV) ejection fraction, LV fractional area change, right ventricular (RV) fractional area change, LVGLS, and RVGLS were significantly reduced (all P < .05), while the RV diameter Z-score, RV/LV diameter, SAS score, and Celermajer index were obviously increased in TVM-R/A in both trimesters (all P < .05). Both LVGLS and RVGLS correlated with cardiothoracic circumference ratio, RV diameter Z-score, RV/LV diameter, Celermajer index, and SAS score (all P < .01). There was a slow decline for LVGLS and RVGLS in TVM-N fetuses throughout the gestation. Conversely, these two parameters worsened rapidly in TVM-R/A group. CONCLUSIONS: TVM fetuses present biventricular dysfunction by deformation analysis in the second and third trimester of pregnancy. TVM fetuses with reduced or absent pulmonary flow exhibit significantly greater impairment and more rapid deterioration of cardiac function, which may contribute to poor outcomes.

Prenatal diagnosis of isolated tricuspid valve dysplasia by echocardiography / 中华超声影像学杂志

Objective:To improve the prenatal recognition and diagnostic accuracy of isolated tricuspid valve dysplasia (TVD) by analyzing fetal echocardiographic features.Methods:The fetal echocardiographic features of 7 cases with prenatal diagnosis of TVD were analyzed retrospectively in Pediatric Cardiovascular Center, Beijing Anzhen Hospital, Capital Medical University and Children′s Hospital of Shanxi from June 2012 to December 2020. The fetal echocardiographic characteristics of TVD were summarized.Results:Among the 7 fetuses with TVD, 6 cases showed functional pulmonary atresia with intact ventricular septum and different degrees of pulmonary valve regurgitation, and 1 case showed slightly narrowed pulmonary artery. Characteristic sonographical findings included: ①The axis of heart was exaggerated left-axis deviation and the ratio of cardiothoracic was obviously increased. The right heart was enlarged, in which the right atrium was significantly dilated (an important feature of TVD). ②The position of tricuspid valve ring was normal, the attachment point of each leaf was normal, some tricuspid valves were thickened and insufficiency. ③It was common to be complicated with functional pulmonary valve atresia with intact ventricular septum or severe pulmonary valve stenosis. ④Color Doppler imaging showed severe tricuspid regurgitation and the regurgitation velocity was less than 350 cm/s (an important feature of TVD). ⑤In fetus with functional pulmonary valve atresia with intact ventricular septum, color Doppler imaging showed retrograded ductal flow across the pulmonary artery and the different degrees regurgitation of pulmonary valve.Conclusions:Fetal TVD has typical echocardiographic features, mainly including large tricuspid regurgitation and significant enlargement of the right atrium. The prognosis of fetus with functional pulmonary atresia is poor. Early diagnosis plays a crucial role in prenatal consultation and prognosis evaluation.

Canine infantile left ventricular noncompaction.

BACKGROUND: Left ventricular noncompaction (LVNC) is a rare form of cardiomyopathy currently described in humans and cats. It consists of a spongy myocardium characterized by prominent trabeculation and deep recesses involving more than 50% of the ventricular thickness. We describe the clinical and pathological features of LVNC combined with tricuspid valve dysplasia, double-orifice tricuspid valve and severe pulmonary stenosis in a puppy. In addition, we briefly review the LVNC causes, pathogenesis, forms and current diagnostic criteria. CASE PRESENTATION: A seven-week-old intact German Shorthaired Pointer-cross male was presented with a poor body condition, exercise intolerance and dyspnea. Clinical exam identified a bilateral systolic murmur (grade IV/VI over the right heart base and grade III/VI over the left heart base). Echocardiography identified tricuspid valve dysplasia, mild mitral regurgitation, and severe pulmonic stenosis with a trans-valvar systolic pressure gradient of 106 mmHg. Left ventricular noncompaction was diagnosed by necropsy and further confirmed histopathologically by the presence of two distinct myocardial layers: an inner noncompacted zone covering more than 50% of ventricular thickness containing prominent trabeculation and deep recesses, and an outer zone of compact myocardium. CONCLUSIONS: This is the first case describing LVNC in a canine patient, supporting the introduction of this form of heart disease as a differential diagnosis for cardiomyopathies in juvenile and adult dogs.

Fetal echocardiographic prediction score for perinatal mortality in tricuspid valve dysplasia and Ebstein's anomaly.

OBJECTIVES: Tricuspid valve dysplasia (TVD) and Ebstein's anomaly (EA) diagnosed by fetal echocardiography vary greatly in terms of clinical severity and prognosis. The Celermajer index and Simpson-Andrews-Sharland (SAS) score have been reported previously for the prediction of prognosis in cases of TVD/EA; however, they do not take into account the hemodynamic impact of left ventricular (LV) function, which has recently been implicated as being important in the pathophysiology of TVD/EA. The aim of this study was to develop a novel scoring system that includes LV function for the prediction of perinatal death in fetuses diagnosed with TVD/EA. METHODS: The clinical records of 36 fetuses diagnosed prenatally with TVD/EA between 2000 and 2015 in our hospital were reviewed. Univariate analysis was used to assess the association between perinatal death (defined as death between 22 weeks' gestation and 4 weeks after delivery) and gestational age at diagnosis, cardiothoracic area ratio (CTAR), degree of pulmonary artery flow, direction of ductal flow, right-to-left ventricular diameter ratio, tricuspid regurgitation (TR) maximum velocity, Celermajer index, SAS score and LV-Tei index. A new prognostic score, the TRIPP score (TRIcuspid malformation Prognosis Prediction score), was developed using the parameters found to be associated significantly with perinatal death. The predictive value of this score was assessed in an additional nine fetuses diagnosed with TVD/EA. RESULTS: Thirty-six fetuses were diagnosed prenatally with TVD/EA, two of which were terminated, one was lost to follow-up and two died before 22 weeks' gestation. Of the 31 included fetuses, 10 (32%) died in the perinatal period. Univariate analysis demonstrated that TR maximum velocity was significantly lower (2.22 ± 0.17 m/s vs 3.26 ± 0.12 m/s; P < 0.001) and SAS score was significantly higher (5.7 ± 0.6 points vs 2.8 ± 0.4 points; P = 0.0014) in cases of perinatal death than in surviving fetuses. The degree of pulmonary artery flow and the direction of ductal flow were also associated significantly with perinatal death (P < 0.01 for both). Notably, LV-Tei index was significantly higher in cases of perinatal death than in surviving fetuses (0.81 ± 0.08 vs 0.50 ± 0.05; P < 0.001). In contrast, there was no significant difference in Celermajer index, CTAR or right-to-left ventricular diameter ratio. Finally, we established a novel combinatorial scoring system, the TRIPP score, including the four significant factors: TR maximum velocity, pulmonary artery flow, direction of ductal flow and LV-Tei index. The TRIPP score was found to predict efficiently perinatal mortality in fetuses with TVD/EA. CONCLUSIONS: Our novel combinatorial score of echocardiographic parameters, the TRIPP score, including LV-Tei index, is easy to measure and provides a good tool for the prediction of perinatal mortality in fetuses diagnosed prenatally with TVD/EA. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Cor triatriatum dexter in 17 dogs.

OBJECTIVES: The objective of this study was to report the signalment, presentation, clinical and imaging findings, interventions, and outcomes in a group of dogs with cor triatriatum dexter (CTD). ANIMALS: Seventeen client-owned dogs. METHODS: Medical records were reviewed retrospectively for signalment, history, physical examination findings, imaging and diagnostic findings, presence of concurrent congenital cardiac defects, description of interventional procedures, therapy information, and outcomes. RESULTS: Age at presentation ranged from two to 110 months, with 10 of 17 dogs (59%) aged <12 months. There was an equal distribution between the sexes. Peritoneal effusion was the most common presenting complaint, in 10 of 17 dogs (59%). The CTD was an isolated finding in 3 of 17 dogs (18%); the remaining 14 of 17 (82%) dogs had concurrent cardiac disease, with congenital anomalies present in 12 of 17 (70%). All except one of these 12 dogs had at least one additional condition affecting the right heart. Tricuspid valve dysplasia was the most common congenital comorbidity, present in 9 of 17 dogs (53%). Seven dogs (41%) underwent interventional treatment of their CTD. In 7 of 17 (41%) cases, the CTD was considered to be incidental and the dogs were asymptomatic; therefore, no interventions were performed. The remaining three cases were euthanized or lost to follow-up. CONCLUSIONS: Cor triatriatum dexter in dogs is commonly seen in association with other right-sided congenital cardiac anomalies and may be an incidental finding. Dogs with CTD obstructing right atrial inflow can have a good outcome after intervention. Dogs with no clinical signs associated with the CTD may remain asymptomatic into adulthood.

Surgical valvulotomy for tricuspid valve stenosis in a dog.

A 2-year, 4-month-old neutered female Labrador retriever was brought for evaluation of right-sided congestive heart failure. Echocardiographic examination revealed tricuspid valve dysplasia with only two small orifices in the valve resulting in severe tricuspid stenosis. The dog underwent a right fifth lateral intercostal thoracotomy and surgical tricuspid valvulotomy, under cardiopulmonary bypass. The stenosis was relieved by dividing the valve leaflets between the two orifices with continuation to the commissures, creating a 'bileaflet' valve. The dog made a good recovery initially, with echocardiography at 48 h after surgery showing a reduction in tricuspid valve E and A wave velocities and pressure half-time (from 230 ms to 65 ms). She was discharged five days after surgery, and spironolactone, benazepril, pimobendan, and clopidogrel were prescribed. The dog was re-presented two days later having collapsed, with pyrexia, facial swelling, and pitting edema on the ventral neck and intermandibular region. Investigations did not reveal an underlying cause, and the clinical signs resolved with supportive therapy. Two years after surgery, the dog was free of clinical signs with normal exercise tolerance and only mild tricuspid regurgitation on echocardiography, with discontinuation of all medications.

Outcomes of Ebstein's Anomaly Patients Treated with Tricuspid Valvuloplasty or Tricuspid Valve Replacement: Experience of a Single Center.

BACKGROUND: The incidence of Ebstein's anomaly is extremely low, and except for the Mayo Clinic, no cardiac center has reported on a sufficient number of patients. The aim of our study was to report the outcomes of Ebstein's anomaly patients treated with tricuspid valvuloplasty (TVP) or tricuspid valve replacement (TVR). METHODS: TVP or TVR was performed in 245 patients from July 2006 to April 2016. We reviewed patients' records and contacted patients via outpatient service and over the telephone. RESULTS: The mean follow-up time was 43.6 ± 32.6 months, and 224 (91.4%) patients underwent follow-up. The mean operative age was 31.2 ± 15.7 years. TVR was performed in 23 patients, and TVP was performed in 201 patients. The 30-day mortality rate was 1.3%, and the overall survival rate was 97.9% at 5 and 10 years. The early mortality rate of the TVP group was lower than that of the TVR group (0.5% vs. 8.7%, P = 0.028), and the overall mortality rate of the TVP group was lower than that of the TVR group, without statistical significance (1.0% vs. 8.7%). After propensity score matching, the rates of mortality and New York Heart Association class ≥ III were lower in the TVP group than those in the TVR group without statistical significance. Seven patients with Type B Wolff-Parkinson-White (WPW) syndrome underwent one-stage surgery, and arrhythmias disappeared. Six patients suffered from episodes of left ventricular outflow tract obstruction (LVOTO) during surgery. Severe LVOTO could be treated with reoperation of the atrialized right ventricle. CONCLUSIONS: Ebstein's anomaly patients treated with TVP or TVR can experience optimal outcomes with midterm follow-up. However, TVP should be the first-choice treatment. Optimal outcomes can be obtained from one-stage operation in patients with Type B WPW syndrome. Severe LVOTO during surgery might be related to improper operation of the atrialized right ventricle.

Outcomes of Ebstein's Anomaly Patients Treated with Tricuspid Valvuloplasty or Tricuspid Valve Replacement: Experience of a Single Center / 中华医学杂志(英文版)

<p><b>Background</b>The incidence of Ebstein's anomaly is extremely low, and except for the Mayo Clinic, no cardiac center has reported on a sufficient number of patients. The aim of our study was to report the outcomes of Ebstein's anomaly patients treated with tricuspid valvuloplasty (TVP) or tricuspid valve replacement (TVR).</p><p><b>Methods</b>TVP or TVR was performed in 245 patients from July 2006 to April 2016. We reviewed patients' records and contacted patients via outpatient service and over the telephone.</p><p><b>Results</b>The mean follow-up time was 43.6 ± 32.6 months, and 224 (91.4%) patients underwent follow-up. The mean operative age was 31.2 ± 15.7 years. TVR was performed in 23 patients, and TVP was performed in 201 patients. The 30-day mortality rate was 1.3%, and the overall survival rate was 97.9% at 5 and 10 years. The early mortality rate of the TVP group was lower than that of the TVR group (0.5% vs. 8.7%, P = 0.028), and the overall mortality rate of the TVP group was lower than that of the TVR group, without statistical significance (1.0% vs. 8.7%). After propensity score matching, the rates of mortality and New York Heart Association class ≥ III were lower in the TVP group than those in the TVR group without statistical significance. Seven patients with Type B Wolff-Parkinson-White (WPW) syndrome underwent one-stage surgery, and arrhythmias disappeared. Six patients suffered from episodes of left ventricular outflow tract obstruction (LVOTO) during surgery. Severe LVOTO could be treated with reoperation of the atrialized right ventricle.</p><p><b>Conclusions</b>Ebstein's anomaly patients treated with TVP or TVR can experience optimal outcomes with midterm follow-up. However, TVP should be the first-choice treatment. Optimal outcomes can be obtained from one-stage operation in patients with Type B WPW syndrome. Severe LVOTO during surgery might be related to improper operation of the atrialized right ventricle.</p>

Tricuspid valve dysplasia: A retrospective study of clinical features and outcome in dogs in the UK.

The objective of this study was to determine the demographic, clinical and survival characteristics and to identify risk factors for mortality due to tricuspid valve dysplasia in UK dogs. Records of client-owned dogs diagnosed with tricuspid valve dysplasia at a referral centre were retrospectively reviewed. Only dogs diagnosed with tricuspid valve dysplasia based on the presence of a right-sided heart murmur identified prior to one year of age, and confirmed with Doppler echocardiography, were included. Dogs with concomitant cardiac diseases, pulmonary hypertension and/or trivial tricuspid regurgitation were excluded. Analysed data included signalment, reason for presentation, clinical signs, electrocardiographic and echocardiographic features, survival status and cause of death. Survival times and risk factors for mortality were evaluated using Kaplan-Meier curves and Cox regression. Eighteen dogs met inclusion criteria. Border collies were over-represented (p= 0.014). Dogs were most frequently referred for investigation of heart murmur. The most common arrhythmia was atrial fibrillation (n=3). Median survival time from diagnosis of tricuspid valve dysplasia was 2775 days (range 1-3696 days; 95% CI 1542.41-4007.59) and from onset of right-sided congestive heart failure was 181 days (range 1-2130 days; 95% CI 0-455.59). Syncope was the sole risk factor for cardiac death. In this population of UK dogs, tricuspid valve dysplasia was uncommon but, when severe, frequently led to right-sided congestive heart failure. Prognosis was favourable for mild and moderate tricuspid dysplasia. Survival time was reduced with right-sided congestive heart failure but varied widely. Risk of cardiac death was significantly increased if syncope had occurred.

The occurrence and suspected mode of inheritance of congenital subaortic stenosis and tricuspid valve dysplasia in Dogue de Bordeaux dogs.

The Dogue de Bordeaux (DdB) breed has gone through several genetic 'bottle necks' and has a relatively small effective population size. Importing new stock into Israel has been limited, further narrowing the already restricted local gene-pool and increasing the chances of inherited defects. In 56 DdB dogs examined between 2003 and 2010, the authors sought to study the proportion congenital subaortic stenosis (SAS) and tricuspid valve dysplasia (TVD). The aim was also to identify a probable mode of inheritance (MOI) using segregation and pedigree analyses of genealogical data available from 13/21 DdB dogs diagnosed with these conditions between 2004 and 2007. Among all breeds in the country, TVD was highest in the DdB breed, which also displayed the second highest proportion of SAS. Echocardiographic measurements and selected physical examination findings from 26 normal DdB dogs, 18 DdB dogs with SAS, and 12 DdB dogs with TVD are reported. Based on pedigree and segregation analyses, the most probable MOI appeared to be autosomal recessive. Pedigree analyses helped to identify three ancestors that might have introduced these two congenital heart defects into the local DdB population. Excluding those three dogs and their progeny from future mating could therefore reduce the prevalence of these diseases in the DdB population in Israel. The unusual local breeding circumstances may offer a unique opportunity to identify associated SAS and TVD genes in the DdB, as well as in other dog breeds.