A "congenitally corrected" variant of Ebstein's anomaly.

Ebstein's anomaly is a rare CHD that is characterised by caudal displacement of the functional tricuspid annulus and a dysfunctional tricuspid valve owing to a failure of proper leaflet coaptation. We present a balanced variant of Ebstein's anomaly, in which the overgrowth of the septal leaflet had allowed proper coaptation of the tricuspid leaflets, thus preserving the valve function.

Tricuspid Valve Repair in Infancy Using Neochordae: Three-Dimensional Echocardiographic Imaging.

Tricuspid regurgitation (TR) in infancy poses a surgical challenge. Both two- and three-dimensional echocardiography (3DE) can provide detailed information about the mechanism(s) of valve failure and insights into valve adaptation during follow-up. We report two patients who underwent tricuspid valve repair using Gore-Tex neochordae, repairs which were facilitated by and assessed with 3DE. Both infants had less than mild residual TR and no valve tethering at hospital discharge. Furthermore, follow-up 3DEs have helped to confirm valve competence, lack of tethering, and growth of the valve and valve apparatus.

Myocarditis, flail tricuspid valve, and normal rhythm: an exceptional form of neonatal cardiac lupus.

Neonatal cardiac lupus is a rare, passively acquired autoimmune disease. We report a case of in utero myocarditis, confirmed postnatally, with papillary muscle rupture and severe tricuspid regurgitation after birth in the absence of conduction disturbances. Tricuspid repair was successfully performed with polytetrafluoroethylene neochordae. In this article, we discuss the pathophysiology, medical and surgical management, and implications at follow-up in this unique scenario.

Successful replacement of the systemic tricuspid valve with a mechanical valve in a 3-month-old boy with congenitally corrected transposition of the great arteries having a dysplastic tricuspid valve.

There are a few reports of successful replacement of the left-sided systemic tricuspid valve with a mechanical valve in small infants with congenitally corrected transposition of the great arteries having Ebstein's anomaly. Tricuspid valve replacement is the preferred option when pulmonary artery banding, performed as a prelude to performing the double-switch operation, is not feasible because of severe heart failure caused by tricuspid regurgitation.

Contemporary Outcomes and Factors Associated With Mortality After a Fetal or Neonatal Diagnosis of Ebstein Anomaly and Tricuspid Valve Disease.

BACKGROUND: Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) are rare anomalies and data on outcomes after a fetal or neonatal EA/TVD diagnosis are conflicting. METHODS: To examine the outcome and identify markers predictive of mortality, we reviewed our single-centre experience from 2000-2014. Variables were analyzed separately for cases diagnosed in utero without pregnancy termination and for all live-born patients. RESULTS: Of 47 fetal cases, 8 (17%) died in utero and 10 (21%) as neonates. Independent predictors associated with fetal demise included severe tricuspid regurgitation with a Doppler gradient < 40 mm Hg (odds ratio, 1.22 per mm Hg deduction; P = 0.003) and pulmonary regurgitation (odds ratio, 11.4; P = 0.03) at the baseline examination. A novel prognostic score (range, 0-10) combining the severity of 5 echocardiographic findings was independently associated with overall mortality (hazard ratio [HR], 1.39 per point increase; P = 0.01). Survival rates of 66 live births at 1 month, 1 year, and 5 years were 86%, 82%, and 80% respectively, and 75%, 60%, and 55% remained free from surgery at the same points in time. Factors associated with postnatal death in multivariate analysis included a younger gestational age at birth (HR per week, 1.59; P < 0.001), tricuspid annulus diameter (HR per z-score increase, 1.76; P = 0.004), and no pulmonary forward flow (HR, 4.63; P = 0.03). CONCLUSIONS: Our experience with fetal and neonatal EA/TVD shows better survival rates than previously reported. Mortality after a fetal diagnosis was significantly associated with hemodynamic changes indicative of a circular shunt, including pulmonary and tricuspid regurgitation severe enough to cause diastolic umbilical arterial flow reversal.

Double-Orifice Tricuspid Valve: A Case Report and Literature Review.

Duplication of an atrioventricular valve is a rare congenital anomaly that usually involves the mitral rather than the tricuspid valve (TV). Isolated appearances of a double-orifice TV (DOTV) are extremely rare and in most cases are associated with other congenital anomalies. We report a rare case of an HIV-seropositive male with a DOTV, divided right atrium and Wolff-Parkinson-White syndrome admitted to our hospital with palpitations and dyspnea. After medical therapy, the patient's symptoms were alleviated. We summarized the characteristics of the DOTV and reviewed the literature.

Ebstein's anomaly in those surviving to adult life - a single centre experience.

BACKGROUND: Ebstein's anomaly (EA) occurs in about one to five per 200 000 live births. Long-term follow-up data of adults with EA is scarce due to the relatively low frequency of the disease and the variation of its anatomic and haemodynamic severity. METHODS: Since 1995, in our adult congenital heart disease (ACHD) centre, we have practised a uniform approach to management of adults with EA, with surgery reserved for those with refractory arrhythmia (failed medical and/or catheter-based treatment) or worsening symptoms of breathlessness. A retrospective review of medical records of all such patients with EA and normal cardiac connections was performed. RESULTS: Fifty-one EA patients (17 males) were identified. Mean age at diagnosis was 21+/-21 years and mean follow-up time at our centre was 21±14 years. During this time, 18 patients (35%) had documented supraventricular arrhythmia. Sixteen patients (30%) underwent ablation therapy with long-term relief from arrhythmia in nine (56%). Nine patients (18%) underwent tricuspid valve (TV) surgery (four repair and five replacement), with seven patients having undergone a tricuspid valve surgery prior to referral to our unit. Three patients died, one of cardiogenic shock after redo surgery (58 years), one of progressive heart failure (45 years) and one with malignancy. Overall survival was 100% to age 40 years, 95% to age 50 years and 81% to age 60 years. CONCLUSIONS: Ebstein's Anomaly in adulthood often has severe morphological abnormalities but is compatible with good medium-term survival, with a generally symptom driven approach to the indications for interventions.

Percutaneous tricuspid valve implantation: two-center experience with midterm results.

BACKGROUND: Severe tricuspid valve (TV) dysfunction may lead to surgical TV replacement with a biological valve prosthesis in patients with congenital heart disease. To expand the lifetime of this valve and reduce the number of surgeries, percutaneous TV implantation (PTVI) may be an effective alternative to repeated surgery. We report on our 2-center experience with PTVI. METHODS AND RESULTS: Between 2008 and 2014, 17 percutaneous valves were implanted in 16 patients with TV bioprosthesis dysfunction (9 females) from 2 centers. Median age and weight were 31.3 years (5-77.2) and 65.2 kg (17.7-107); 14 patients had congenital heart disease (univentricular heart with a right atrial to right ventricle bioprosthesis in 3, Ebstein's anomaly of the TV in 5, and other in 6), and 2 had acquired TV dysfunction. All procedures were successful (Melody n=7, Sapien 26 mm valve n=4, Sapien XT 29 mm valve n=6). One valve showed early dysfunction. It was replaced surgically and shortly after that a repeated PTVI was performed. The median duration of follow-up was 2.1 years (3 days to 6.3 years). The percutaneous valve was performing well in 15 of 16 patients. CONCLUSIONS: PTVI was safe and effectively improved TV function in all but 1 patient at midterm follow-up. We think that PTVI is a good alternative to repeated surgical TV replacements and that it may reduce the total number of open heart surgeries in these patients.

Anterior and posterior leaflets augmentation to treat tricuspid valve regurgitation.

In congenital non-Ebstein anomalies of the tricuspid valve, the septal leaflet is often involved and tethered. We describe a standardized approach to address septal leaflet tethering by concomitant augmentation of the anterior and posterior leaflets.

First trimester tricuspid regurgitation and fetal abnormalities.

BACKGROUND: Tricuspid regurgitation (TR) is a common sonographic finding during the fetal life. It has been reported in 7% of normal fetuses. It may be associated with aneuploidy and with both cardiac and extracardiac defects. OBJECTIVES: In this study, we have looked at the characteristics of fetuses with and without TR at 11+0 to 13+6 weeks' gestation. Groups were compared with respect to the following variables: prevalence of chromosomal defects; other markers of aneuploidy; fetal cardiac; and extracardiac anomalies. METHODS: The study group included women, who underwent an ultrasound examination at 11-13+6 weeks' gestation between 2009 and 2012. The inclusion criteria were singleton pregnancies with crown-rump length measurements of 45-84 mm where the pregnancy outcome was known. RESULTS: Some 1075 patients met our inclusion criteria including 979 fetuses without TR and 96 with TR. There were 72 cases of aneuploidy diagnosed (6.7%). Isolated TR was found in 53 euploid fetuses (5.2%). All of the TR(+) aneuploid fetuses (n=40) had additional ultrasound markers present. Extracardiac anatomy showed a higher prevalence of abnormalities in the group of TR positives (12.5%) vs. TR negatives (1.6%). Abnormal cardiac findings were more frequent in the TR-positive group independently of chromosomal status and were found in 18.8% of fetuses with TR and in 1.9% with a normal tricuspid flow. CONCLUSIONS: TR in combination with other markers is the strongest predictor for aneuploidy. TR, as an isolated parameter, is a poor screening tool both for all and for each individual chromosomal abnormality and congenital cardiac defects.

Tricuspid annulus: a three-dimensional deconstruction and reconstruction.

BACKGROUND: Before clinical manifestation of regurgitation, the tricuspid annulus dilates and flattens when right ventricular dysfunction is potentially reversible. That makes the case for a prophylactic tricuspid annuloplasty even in the absence of significant tricuspid regurgitation. Owing to the appreciation of the favorable prognostic value of tricuspid annuloplasty, the geometry of the normal tricuspid annulus merits critical analysis. METHODS: Three-dimensional transesophageal echocardiographic data from 26 patients were analyzed using Image Arena (TomTec, Munich, Germany) software. Cartesian coordinate data from tricuspid annuli were exported to MATLAB (Mathworks, Natick, MA) for further processing. Annular metrics related to size, shape, and motion were computed. RESULTS: The tricuspid annulus demonstrated significant changes in area (p<0.01) and perimeter (p<0.03) during the cardiac cycle, with maximum values attained at end diastole. There was significant correlation between two- and three-dimensional area changes, indicating true expansion in the annulus. The anterolateral region of the annulus demonstrated the greatest dynamism (p<0.01), and the anteroseptal region showed the least. The anteroseptal region also displayed the most nonplanarity in the annulus. In addition, vertical translational motion was observed, with a mean distance of 11.3±3.7 mm between end systolic and end diastolic annular centroids. CONCLUSIONS: The tricuspid annulus is a dynamic, multiplanar structure with heterogeneous regional behavior. These characteristics should be taken into account for optimal annuloplasty device design and efficacy.

Use of pimobendan in feline congenital heart failure.

A 6-month-old domestic shorthair cat was referred for evaluation of sudden lethargy and tachypnea following ovariohysterectomy. Upon failure of improvement with supportive care, a cardiologist identified congenital tricuspid dysplasia with signs of heart failure. Furosemide, enalapril, and pimobendan were used to reduce clinical signs and improve length and quality of life.

Usage de pimobendane lors d'une insuffisance cardiaque féline congénitale. Un chat commun domestique âgé de 6 mois a été recommandé pour l'évaluation d'un abattement et d'une tachypnée d'apparition soudaine après l'ovariohystérectomie. En absence d'amélioration avec des soins de soutien, un cardiologue a identifié une dysplasie tricuspidienne congénitale avec des signes d'insuffisance cardiaque. Le furosémide, l'enalapril et le pimobendane ont été utilisés pour atténuer les signes cliniques et améliorer la durée et la qualité de vie.(Traduit par Isabelle Vallières).

Long-term results of modified Fontan operation for single-ventricle patients associated with atrioventricular valve regurgitation.

BACKGROUND: The long-term outcome of modified Fontan operation concomitant with a valve operation for atrioventricular valve (AVV) regurgitation is not well described. METHODS: Between 1977 and 2003, 500 children who underwent modified Fontan operation were subdivided into 192 with AVV plasty (group P) and 308 without AVV plasty (group N). Factors associated with patient outcome were investigated retrospectively. RESULTS: Surgical techniques to correct valve incompetence included circular annuloplasty, partial annuloplasty with Kaye-Reed methods, edge-to-edge repair methods, and valvoplasty, which were combined according to the etiology of the valve lesion. The estimated actuarial survival rates at 10 and 20 years were, respectively, 82.0% and 76.6% in group P (p < 0.05) and 90.8% and 86.8% in group N (p = 0.001). The estimated actuarial survival rates at 10 years among patients with AVV plasty did not show a statistically significant difference (circular annuloplasty, 79.0%; partial annuloplasty, 81.6%; edge-to-edge, 83.3%; valvoplasty, 82.6%; p = 0.90). A Cox regression model revealed that a higher preoperative ventricular ejection fraction was associated with a significant reduction in long-term mortality in group P (hazard ratio, 0.921; 95% confidence interval, 0.873 to 0.972; p < 0.05). Each repair method showed acceptable durability at medium-term follow-up, without progression of lesions. CONCLUSIONS: Given worse results with poor ventricular function, early intervention against valve pathology before development of ventricular failure may improve long-term outcomes. Multiple methods are appropriate for a variety of valve lesions; however, circular annuloplasty remains a reliable repair option due to its technical simplicity.

[Screening performance of first trimester nuchal translucency, ductus venosus blood flow and tricuspid regurgitation for cardiac defects]. / Fetale Nackentransparenz und weitere Ersttrimester Marker: Screening auf fetale Herzfehler.

The present article summarises current knowledge on the risk of fetal cardiac malformations in fetuses with increased nuchal translucency (NT), abnormal ductus venosus blood flow pattern and tricuspid regurgitation. The risk of congenital heart disease (CHD) increases with increasing NT. However, nuchal translucency screening is only modestly efficient in the detection of congenital heart disease in low-risk pregnancies. If the nuchal translucency is normal, abnormal ductus venosus blood flow and tricuspid regurgitation show low sensitivities as single screening parameters. The combination of all 3 markers might increase the detection rates. Fetal echocardiography at 20 weeks of gestation is recommended in foetuses with an NT ≥95th percentile, as the incidence of CHD increases 2.5-fold. When the NT measurement is ≥99th percentile, the risk of CHD increases 10-fold. In this high-risk group and in fetuses with tricuspid regurgitation and/or an abnormal DV flow pattern along with an increased NT, first or early second trimester echocardiography performed by experts is warranted. The early examination of the fetal heart should be completed by a detailed echocardiogram at around 20 weeks of gestation.

Modified Starnes procedure in a neonate with severe tricuspid regurgitation.

We report a modification of the Starnes technique for palliating severe tricuspid regurgitation associated with a dysplastic right ventricle in a neonate, using a fenestrated pericardial patch allowing for unidirectional flow. The patient eventually underwent a successful Glenn shunt construction with a persistent reduction in right ventricle size at 1 year follow-up.

Bioprosthesis valve replacement in dogs with congenital tricuspid valve dysplasia: technique and outcome.

OBJECTIVE: To describe the surgical technique and report outcome of dogs undergoing bioprosthesis valve replacement for severe tricuspid regurgitation (TR) secondary to congenital tricuspid valve dysplasia (TVD). ANIMALS, MATERIALS AND METHODS: Twelve client-owned dogs (19-43 kg) with TVD underwent tricuspid valve replacement with a bovine pericardial or porcine aortic bioprosthesis with the aid of cardiopulmonary bypass. Anticoagulation with warfarin was maintained for 3 months after surgery and then discontinued. RESULTS: Ten of 12 (83.3%) dogs survived surgery and were discharged from the hospital. Seven dogs were alive with complete resolution of TR for a median period of 48 months (range 1-66 months) after surgery. Two dogs underwent euthanasia because of bioprosthesis failure due to inflammatory pannus at 10 and 13 months after surgery. Two dogs experienced valve thrombosis that was resolved by tissue plasminogen activator. One dog developed suspected endocarditis after surgery that was resolved with antibiotics. Serious cardiac complications included atrial fibrillation and flutter, right-to-left shunt through an uncorrected patent foramen ovale, complete atrioventricular block, and sudden cardiac arrest. Postoperative atrial fibrillation or flutter did not occur in 7 dogs treated prophylactically with oral amiodarone before surgery. CONCLUSIONS: Curative intermediate-term outcomes are possible in dogs undergoing open tricuspid valve replacement with a bioprosthesis. Prosthesis-related complications include inflammatory pannus, thrombosis, and endocarditis. Postoperative atrial fibrillation or flutter can be reduced or prevented by prophylactic preoperative treatment with amiodarone. Several identified complications are avoidable or can be reduced with increased awareness and experience with these techniques.