Unmasking pulmonary hypertension following tricuspid valve replacement

In this Viewpoint, we highlight a possible hemodynamic problem arising following tricuspid valve replacement (TVR) in patients with severe chronic tricuspid regurgitation, represented by “unmasking” of pulmonary hypertension (PH) following the surgery. We share an observation that should alert cardiologists to the fact that this increasingly utilized surgery is not risk free, and careful assessment of the right ventricular function and pulmonary circulation preoperatively is extremely important, especially in patients with preexisting risk factors for PH, since TVR may lead to a sudden increase in right ventricular afterload.     

Melody valve implantation in the pulmonary and tricuspid position

Percutaneous implantation of valved stents is now routinely performed to treat pulmonary valve regurgitation and stenosis. In addition, there are isolated reports of implantation of valved stents in the tricuspid position to treat prosthetic tricuspid stenosis or regurgitation when a prosthetic valve ring exists. We present a case in which a patient with combined severe tricuspid valve and pulmonary valve disease was successfully treated with sequential implantation of percutaneous valved stents in a single procedure. The procedure was straightforward, of short duration, and dramatically improved the patient's functional status. © 2012 Wiley Periodicals, Inc.     

Isolated rheumatic severe tricuspid regurgitation

Severe isolated tricuspid regurgitation (TR) is very rare, with most cases of TR being functional and secondary to pulmonary hypertension from left heart pathologies. We report an unusual case of a young Nigerian male, who presented to us with dyspnea, repeated hospital admissions for heart failure, and a childhood history of rheumatic fever. Echocardiogram showed massively dilated right atrium and ventricle, noncoaptation of thickened tricuspid valve with torrential free tricuspid regurgitation. Other valves were normal. Cardiac MRI showed normal right ventricular function and viability. Patient underwent tricuspid valve replacement with 35‐mm St. Jude valve.     

A case of congenital tricuspid valve abnormality showing six leaflets

A-59-year old woman died from heart failure associated withtricuspid regurgitation and pulmonary hypertension.Necropsyrevealed that her tricuspid valve was composed of six leaflets.We found no previous report of this type of tricuspid valveabnormality.     

Transcatheter tricuspid valve replacement With the Edwards SAPIEN valve

We describe a case of percutaneous tricuspid valve implantation in a 20‐year‐old man with previous tricuspid valve replacement in the setting of pulmonary atresia with intact ventricular septum. He developed symptomatic endocarditis‐induced tricuspid regurgitation of the tricuspid bioprosthesis. Tricuspid valvar competence was restored with implantation of a 26‐mm Edwards SAPIEN valve. © 2011 Wiley‐Liss, Inc.     

Is right atrioventricular valve always tricuspid?

A 71‐year‐old male with a medical history of hypertension was admitted to emergency department with hypertensive pulmonary edema. The patient appeared anxious and diaphoretic, and physical examination revealed tachypnea, crepitant rales in the both lungs and a systolic ejection murmur at the right sternal border, radiating to the both carotid arteries. The electrocardiography showed sinus tachycardia with indications of left ventricle hypertrophy Before the aortic valve surgery, transesophageal echocardiography (TEE) was performed for detailed evaluation of the aortic valve. Two‐dimensional TEE showed mild tricuspid valve regurgitation and only two of three leaflets of the tricuspid valve at a time.     

Pulmonary atresia with an intact interventricular septum and Ebstein's anomaly of the tricuspid valve

Patients with pulmonary atresia and intact ventricular septum have a poor prognosis with or without conventional surgical treatment. The best results of surgical treatment are obtained in those cases with a mild underdeveloped right ventricle and minor sinusoidal communication in the absence of important dysfunction of the tricuspid valve. We present five cases of pulmonary atresia with intact ventricular septum associated with dysfunction of the tricuspid valve. On the basis of radiographic, electrocardiographic and hemodynamic findings, this group of patients could not be distinguished from others without dysplasia of the tricuspid valve. Echocardiographic and angiocardiographic studies are mandatory in the differential diagnosis. A combination of systemic-pulmonary artery anastomosis associated with pulmonary valvotomy, when possible, and reconstruction of the right ventricular outflow tract are indicated for surgical solution of these malformations. However, tricuspid valve replacement is indicated in some cases.     

Imperforate tricuspid valve with single cardiac outlet from right ventricle

A cyanosed neonate was diagnosed as having concordant atrioventricular connection and single cardiac outlet and aorta from right ventricle with atresia of the pulmonary valve. She underwent a systemic to pulmonary artery shunt and is now thriving. Additionally, the tricuspid valve was imperforate and a large ventricular septal defect caused an unusual pattern of intracardiac blood flow. The tricuspid valve ring and right ventricular cavity appeared to be of normal size. The good outcome in this patient suggests that these features may be advantageous for corrective operation.     

A case of unguarded tricuspid valve orifice diagnosed accidentally in an adult

Unguarded tricuspid orifice is the most extreme of tricuspid valve dysplasia with a very variable natural history. They can tolerate tricuspid regurgitation well, and they become symptomatic only if significant right ventricular dysfunction or atrial fibrillation occurs. Patients with a mild degree of right ventricular dysfunction can survive to adulthood and even reach old age. Surgical treatment is a difficult option due to variable natural history, and surgical results are not too encouraging.     

Imperforate tricuspid valve with single cardiac outlet from right ventricle.

A cyanosed neonate was diagnosed as having concordant atrioventricular connection and single cardiac outlet and aorta from right ventricle with atresia of the pulmonary valve. She underwent a systemic to pulmonary artery shunt and is now thriving. Additionally, the tricuspid valve was imperforate and a large ventricular septal defect caused an unusual pattern of intracardiac blood flow. The tricuspid valve ring and right ventricular cavity appeared to be of normal size. The good outcome in this patient suggests that these features may be advantageous for corrective operation.     

Simulated tricuspid valve echoes in tricuspid atresia

A case of tricuspid atresia with unusual echocardiographic findings is presented. The echocardiogram was successful in defining great artery interrelationships, ventricular looping, and cavity sizes. An echo pattern resembling the motion of a small tricuspid valve was observed; this was proven to be spurious at catheterization and necropsy. Caution should be exercised in diagnosing the presence of a tricuspid valve on the basis of atypical echoes from the tricuspid valve area.     

Iatrogenic right coronary artery stenosis resulting from surgical tricuspid valve replacement

Iatrogenic injury to the right coronary artery (RCA) is a rare complication of tricuspid valve surgery. We herein describe the first‐ever report of RCA injury related to tricuspid valve replacement surgery. A 38‐year‐old man with recurrent tricuspid endocarditis underwent redo tricuspid valve replacement by means of a minimally invasive right thoracotomy with a 32‐mm St. Jude bioprosthetic valve. His post‐operative course was complicated by pulseless ventricular tachycardia requiring CPR and defibrillation. Cardiac catheterization revealed a “kinked” stenotic distal RCA. The lesion was noted to be flow limiting by fractional flow reserve and was treated with two everolimus‐eluting stents. The RCA runs in the atrioventricular groove and is susceptible to injury especially in the region of the posterior leaflet of the tricuspid valve, where the relationship of the tricuspid annulus to the RCA is most intimate. Repair of surgically induced coronary stenosis can be accomplished with percutaneous intervention. © 2014 Wiley Periodicals, Inc.     

Straddling tricuspid valve without a ventricular septal defect

A four year old girl with pulmonary atresia had a straddling tricuspid valve without an interventricular communication. The overriding tricuspid valve had two orifices, which connected with the right and the left ventricles. Valve tissue separated both orifices and was firmly connected to the crest of the ventricular septum, thus sealing off the expected interventricular communication. Surgical correction was performed and the outcome was satisfactory.     

Straddling tricuspid valve without a ventricular septal defect.

A four year old girl with pulmonary atresia had a straddling tricuspid valve without an interventricular communication. The overriding tricuspid valve had two orifices, which connected with the right and the left ventricles. Valve tissue separated both orifices and was firmly connected to the crest of the ventricular septum, thus sealing off the expected interventricular communication. Surgical correction was performed and the outcome was satisfactory.     

Cardiomyopathy presenting prenatally with functional tricuspid and pulmonary atresia

Cardiomyopathy is a rare diagnosis in the fetus that usually presents as a dilated, poorly functioning ventricle. We present the case of a fetus that developed functional tricuspid and pulmonary atresia due to progressive right ventricular cardiomyopathy. The baby was supported with prostaglandin and inotropic infusions after delivery, eventually weaning off without need for surgical intervention. This case illustrates the prenatal findings that evolved and the successful postnatal management.     

Congenital pulmonary atresia with tricuspid insufficiency: Morphologic study

In an anatomic study of 21 cases of pulmonary atresia with tricuspid insufficiency (pulmonary atresia with intact ventricular septum, type II), the morphologic features of the tricuspid valve and the right ventricle were found to differ greatly from those seen in pulmonary atresia with tricuspid stenosis (pulmonary atresia with intact ventricular septum, type I). Morphologically, pulmonary atresia with tricuspid insufficiency (type II) has a greater resemblance to Ebstein's disease with pulmonary atresia than to type I pulmonary atresia. The anomaly may be more amenable to surgery than pulmonary atresia with tricuspid stenosis because the right ventricle in the former may be converted into a functional chamber by a valvotomy combined with a shunting procedure and atrial septostomy.     

Assessment of the tricuspid valve morphology by transthoracic real-time-3D-echocardiography

AIM: To demonstrate the feasibility of transthoracic three-dimensional real-time echocardiography (3D-TTE) supplemental to routine assessments of the tricuspid valve and to analyze interrater agreement. METHODS: Twenty healthy subjects and 74 patients with right ventricular failure were examined with conventional 2D and additionally 3D-TTE (SONOS 7500, Philips, Netherlands). The 3D exams were performed and recorded by one of two raters. The recordings were evaluated offline and independently by both raters for visualization of morphological and functional features of the tricuspid valve according to a subjective 3-point scale. Statistical analyses were performed for interrater agreement and for comparison of imaging quality between the two study groups. In addition, we present an illustrative case report. RESULTS: Visualization of the spatial relationship between the tricuspid valve and vicinal structures, of the commissures, the orifice, and entirety of valve depiction were better in the ventricular failure group as compared to the control group. Annular dimensions were equally assessable in both groups, leaflet thickness and mobility were not significantly different. Interrater agreement on assessability was slight for leaflet thickness, fair for leaflet mobility and orifice area, and good for the remaining features. The 3D-TTE exam including offline evaluation took 6.5 minutes on average and maximally 14 minutes. CONCLUSION: 3D-TTE of the tricuspid valve can be performed in addition to routine 2D echocardiography within a reasonable time and with high assessability of important features in patients with right ventricular failure. Interrater agreement was fair to good overall. Thus, its feasibility may encourage prospective studies on its potential for more detailed noninvasive diagnosis and preoperative planning.     

Severe tricuspid stenosis presenting as tricuspid atresia. Echocargraphic diagnosis and surgical management.

Two cases of pulmonary atresia with intact ventricular septum and severe tricuspid stenosis are described in which the initial angiographic diagnosis was tricuspid atresia and pulmonary atresia. Two dimensional echocardiography showed the features of an imperforate tricuspid valve because in each case contrast echocardiography failed to show anterograde flow from the right atrium to right ventricle. Successful radical surgical repair was achieved in one patient with performing a tricuspid valvotomy and inserting an external valved conduit between the hypoplastic right ventricle and the main pulmonary artery. In the second case, an infant died four weeks after tricuspid valvotomy and right ventricular outflow tract reconstruction.     

Three-dimensional echocardiography for the assessment of the tricuspid valve

Tricuspid valve pathology is increasingly recognized as an important contributor to patient morbidity. Accordingly, interest in transcatheter interventions for tricuspid valve disease has continued to grow. Echocardiographic imaging of the tricuspid valve has therefore become an integral component of patient assessment and the essential imaging modality for interventional procedures. The need for improved tricuspid valve imaging has highlighted the variability in tricuspid valve anatomy and the difficulties of using two-dimensional (2D) echocardiography alone to determine the location and type of tricuspid valve disease. Here, three-dimensional (3D) imaging using tools such as biplane imaging, multiplanar reconstruction and live 3D acquisition allow a more accurate and efficient evaluation of the tricuspid valve. The 3D imaging of the tricuspid valve is often focused on transesophageal echocardiography, but the more anterior location of the tricuspid valve also lends itself to assessment with transthoracic echocardiography. In this review, we will examine how 3D imaging can complement and enhance the information obtained from 2D echocardiography, and present novel applications for the quantitation of valvular disease and its utility in intraprocedural imaging.     

Transcatheter tricuspid valve‐in‐valve replacement: Making it simpler

Transcatheter tricuspid valve‐in‐valve replacement (TTVR) represents an attractive therapeutic option in very high surgical risk patients with degenerated tricuspid bioprostheses. However, the procedural management of these patients might be challenging due to their comorbidities, long lasting heart valve disease and the presence of left‐sided mechanical prostheses. Thus, a more “minimally disruptive” procedure would have several potential benefits in such a frail population. We present the cases of four patients admitted for congestive heart failure due to severe degeneration of their tricuspid bioprostheses who were treated with a minimalist TTVR. The protocol combined continuation of the oral anticoagulant treatment with no bridging therapy, the use of a single venous access with local anesthesia, rapid pacing via the stiff right ventricular wire, and transthoracic echocardiographic guidance and assessment, with no need for contrast injection. Our protocol aims at simplifying the TTVR procedure and might be a useful tool to avoid procedural complications and reduce hospital stay.