A Rare Case of Prolapsing Accessory Tricuspid Valve Tissue into the Ventricular Septal Defect in Patient Undergoing Tetralogy of Fallot Repair: Role of Transesophageal Echocardiography
1,2,5Department of Anesthesia and Intensive Care, Postgraduate Institute of Medical Education and Research, Chandigarh, India
3Department of Anesthesia, Yashwant Parmar Medical College, Nahan, Himachal Pradesh, India
4Department of Cardiothoracic and Vascular Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India
Corresponding Author: Sunder Lal Negi, Department of Anesthesia and Intensive Care, Postgraduate Institute of Medical Education and Research, Chandigarh, India, Phone: +91 9888446388, e-mail: firstname.lastname@example.org
How to cite this article Negi SL, Niyogi SG, Norbu S, et al. A Rare Case of Prolapsing Accessory Tricuspid Valve Tissue Into the Ventricular Septal Defect in Patient Undergoing Tetralogy of Fallot Repair: Role of Transesophageal Echocardiography. J Perioper Echocardiogr 2020;8(1):3–5.
Source of support: Nil
Conflict of interest: None
Accessory tricuspid valve tissue (ATV) is a rare congenital cardiac malformation most commonly associated with congenital cardiac anomalies. In literature, reported cases are associated with occlusion of the ventricular septal defect (VSD) by ATV. Therefore, accurate diagnosis of ATV in patients undergoing cardiac surgery is important. We report a rare case of ATV tissue in a patient undergoing tetralogy of Fallot (TOF) repair.
Introduction: Accessory tricuspid valve tissue is a rare congenital cardiac anomaly most commonly associated with congenital cardiac anomalies with varying clinical and hemodynamic features. Most of the reported cases were associated with various other intracardiac anomalies, such as TOF, transposition of a great artery, and VSD. When associated with VSDs, it can cause partial to near-complete obstruction of the VSD or it can cause right ventricular outflow tract (RVOT) obstruction. Therefore, preprocedure diagnosis and confirmation are needed when it is associated with congenital cardiac abnormalities.
Keywords: Accessory tricuspid valve tissue, Tetralogy of Fallot, Transesophageal echocardiography.
A 3-year-old male child presented with cyanosis and short of breath. On examination, the child was hemodynamically stable with a heart rate of 90/minute, regular and blood pressure was 90/58 mm Hg. Room air arterial oxygen saturation was maintained between 70% and 75%. All laboratory parameters were within normal limits. Preoperative two-dimensional (2D) transthoracic echocardiography (TTE) revealed a large perimembranous ventricular septal defect (VSD) with severe infundibular obstruction with 20% aortic override.1–6
In the operating room, standard monitoring was attached and the child was induced with inhalational sevoflurane. Intravenous (IV) cannula was secured in the left upper limb with 20-gauge cannula and injection fentanyl 2 μg/kg, and injection vecuronium 1.5 mg were administered for analgesia and muscle relaxation, respectively. The airway was secured with a 5-mm uncuffed tube and fixed at 13 cm. After full desufflation of the stomach, transesophageal echocardiography (TEE) was introduced. Transesophageal echocardiography probe used was GE Health care Vivid E9 machine, 6VT probe (Norway). Surprisingly, TEE revealed a freely mobile membrane-like tissue originating below the septal leaflet of the tricuspid valve bulging across the septal defect, 11 mm perimembranous VSD with the right to left shunt, narrowing of infundibulum (Figs 1 and 2). The peak flow gradient across the VSD was 8 mm Hg (Figs 3 and 4). The new finding of accessory tricuspid valve tissue (ATV) tissue was conveyed to the surgeon.
After achieving activated clotting time (ACT) of >480 seconds, the child was put on CPB, and under mild hypothermia, infundibulum was resected, VSD was closed with polytetrafluoroethylene (PTFE) patch and accessory valve tissue was surgically excised. CPB and aortic cross-clamp time were 100 and 75 minutes, respectively. Came off bypass on inotropic support of milrinone 0.3 μg/kg/minute and noradrenaline 0.05 μg/kg/minute. The postoperative TEE showed no residual shunt (Fig. 5) and trivial tricuspid regurgitation. The patient was shifted to the cardiac surgical intensive care unit (ICU) and extubated after 12 hours of mechanical ventilation. Intensive care unit stay was uneventful, shifted to ward on day 4, and discharged from hospital on day 7.
Accessory tricuspid valve tissue is a rare cardiac abnormality that is often associated with underlying congenital heart defects, such as tetralogy of Fallot (TOF), transposition of the great arteries (TGA), and VSD.1–6 Therefore, diagnosis of ATV may be missed during echocardiography.
Morphologically, there are two types of ATV that have been described mobile and fixed ATV.1 The mobile type is a freely floating tissue in the right ventricle or outflow tract, as seen in our patient. The fixed type is firmly attached to the interventricular septum by short chordae. Depending on the association of ATV with underlying cardiac defect, it can cause mild to severe obstruction of VSD when associated with VSD.
With the mobile type of ATV, it can alter the presenting symptoms in patients with VSD where the shunt is predominantly left to right, and there is a more chance of right ventricular outflow tract (RVOT) obstruction or it may cause the restriction of VSD.5,6 In our index case, the ATV was restricting the VSD. The diagnosis can be made by echocardiography, computed tomography angiogram, and cardiac magnetic resonance imaging. Two-dimensional TTE usually7 facilitates the diagnosis of the accessory valve tissue but when ATV is associated with complex heart disease this can be missed. Therefore, TEE plays an important role in the perioperative period due to its proximity to underlying cardiac structures.
The ATV should be surgically excised, since it may cause RVOT obstruction.1 The implications of this accessory valve tissue in the perioperative period are very important because in an arrested heart it will be difficult to differentiate between normal and accessory valve tissue. If this accessory tissue does not get excised during surgery it may cause RVOT obstruction which may require going back on CPB a second time to excise this valve tissue. Therefore, accurate perioperative diagnosis is paramount important which can be accomplished by TEE like in our case.
Accessory tricuspid valve tissue is a rare cardiac anomaly but it is an important cause of RVOT obstruction if it is left unaddressed while performing underlying other cardiac anomalies. Transesophageal echocardiography is considered an important imaging modality in the perioperative period because of its minimally invasive nature. Transesophageal echocardiography is indicated in open cardiac surgery; it can be helpful not only in confirming diagnosis but also helpful in diagnosing additional findings because of its proximity to underlying cardiac structures.
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2. Yoon A, Shaqra H, Bella JN, et al. Accessory tricuspid valve leaflet in an asymptomatic adult. Tex Heart Inst J 2008;35(3):327–328.
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7. Eiriksson H, Midgley FM, Karr SS, et al. Role of echocardiography in the diagnosis and surgical management of accessory mitral valve tissue causing left ventricular outflow tract obstruction. J Am Soc Echocardiogr 1995;8(1):105–107. DOI: 10.1016/s0894-7317(05)80367-0.
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