Case Report
Cor Triatriatum Dexter
Khiem Phan*
Corresponding Author: Khiem Phan, Department of Internal Medicine Leonard J. Chabert Medical Center, 1978 Industrial Blvd, Houma, LA 70363, United States.
Received: August 25, 2024; Revised: August 31, 2024; Accepted: September 03, 2024 Available Online: September 20, 2024
Citation: Phan K. (2024) Cor Triatriatum Dexter. Int J Surg Invasive Procedures, 7(3): 189-193.
Copyrights: ©2024 Phan K. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Share :
  • 50

    Views & Citations
  • 10

    Likes & Shares
Cor Triatriatum Dexter (CTD) is a rear heart condition that affects the right atrium. It occurs when a thin membrane emerges and separates the right atrium into two chambers. The medical practitioners can identify the condition during childhood or adulthood, depending on the clinical manifestation. The two compartments make the right atrium more prominent than the left atrium, which will restrict the flow of oxygenated blood due to the closure of atrial septal defect (ASD). The case report shows the CTD for the 40-year-old may not have been diagnosed, resulting from a lack of insurance to access care. The patient was non-compliant with the cardiac diet, taking two bottles of alcohol daily, which may make the condition chronic. The factors could be causing the right atrium and ventricle to deflate unevenly, affecting the normal systolic function. Fluid buildup indicates the end stages of a heart condition, and the correct diagnosis is needed to reduce the effects on the individual. Comprehensive echocardiography and percutaneous catheterization are necessary to eliminate the heart's obstruction and increase the patient's survival rate.

Keywords
: Atrial septal defect (ASD), Cor Triatriatum Dexter (CTD), Echocardiography, Fluid buildup, Percutaneous catheterization, Right atrium
INTRODUCTION

Cor Triatriatum Dexter (CTD) is a heart defect that affects the right or left atrium. Typically, the heart is subdivided into two parts, but people with Cor triatriatum dexter have three atrial chambers due to the thin membrane separating the two main atriums. Cor triatriatum is estimated at around 0.025% of all congenital heart diseases [1]. High percentages of clinical diagnoses show that the rare condition occurs on the embryonic right valve, leading to the right atrium subdivision. The clinical signs of the abnormality may not be present in a patient; it all depends on the degree of obstruction. Cor triatriatum dexter accounts for 0.1% to 0.4% of all infants with congenital heart disease [2]. The condition is usually diagnosed in the first year of life. Sometimes, the disease is not diagnosed until adulthood. Herein, I present a case report of CTD.

Figure 1 shows a case of Incomplete Cor triatriatum dexter. Considering that there are two compartments in the right atrium which are more prominent, there will be a restricted flow of oxygenated blood. The right ventricle will receive limited blood and oxygen because of the involution in the right atrium. That causes a significant amount of abnormalities. The image shows the complications that occur due to percutaneous closure of atrial septal defect (ASD), and disk insertion help offer greater rigidity and traction maneuvers [3].

22Surgical correction can help in reducing the risks of the abnormality. CT is a hereditary abnormality in which an impeding membrane divides the right (dexter) or left (sinister) atrium into two compartments. Cor triatriatum dexter, the rarer of the two disorders, is identified nearly entirely during embryonic stage using echocardiography and may be related with pulmonary artery stenosis, tricuspid abnormalities, or hypoplastic right ventricle [4]. Echocardiography is fundamental in diagnosing the condition. The right atrium's degree of separation can help enhance clinical manifestation. Cor triatriatum dexter is associated or isolated from other heart conditions.

Figure 2 shows the echocardiography images of a neonate with Cor triatriatum dexter. The blue color in the picture signifies the systemic venous blood that is taken back to the right atrium [5].

On the other hand, the red color represents the blood flow on the right atrium's outer part. The diagnosis process is critical to help establish the treatment options that a child or adult suffering from Cor triatriatum dexter will receive.


CASE REPORT

The patient was an immigrant in his 40s, repeatedly admitted to the hospital over the last ten years with a past medical history of congenital heart disease cor triatriaum dexter, abnormal echocardiography of the heart, permanent atrial fibrillation, and chronic heart failure. Transesophageal echocardiogram found small patent foramen ovale (PFO), possibly unroofed coronary sinus with connection to left superior vena cava (SVC). The patient reported that he often had bilateral upper and bilateral lower extremity edema. He denied syncope, dizziness, palpitations, orthopnea, and shortness of breath. The patient got cardiology consults at every admission, but no surgical procedure has been intervened. His BNP was 650, and troponin I was < 0.006. His symptoms, rate, rhythm control, anticoagulation, and thrombotic prophylaxis were treated and get discharged every time. He was compliant with his home medications at home. However, he was non-compliant with his cardiac diet. In addition, he drank 1-2 beers daily. The patient reported he had been sick since childhood but has not had any history of surgical procedure intervention for his heart condition since the patient doesn’t have health insurance. The individual was transferred to other healthcare facilities for further consults and surgery evaluation, but the family could not afford pre-op testing due to financial hardship. Therefore, the patient has been repeatedly admitted here to manage his chronic heart failure symptoms with aspirin (ECOTRIN), 81 MG EC tablet, digoxin (LANOXIN) 125 mcg tablet, furosemide (LASIX) 40 MG tablet, enoxaparin, acetaminophen tablet 650 mg, furosemide injection 40 mg.

DISCUSSION

Cor triatriatum (CT) is a sporadic defect in which the right atrium is separated into binary compartments by a thin, fibromuscular membrane causing division of either the left or right atrium, contributing to the presence of three atrial compartments [6]. In the 4th week of embryonic growth, the atrium is separated from the venous sinus by a valve with two flaps (the venous sinus valve). During the embryo's maturation, the left leaflet of the venous sinus valve is merged into the interatrial septum and is made up of a component of the septum secundum. As the growth occurs, the right leaflet is reabsorbed around the twelfth week of gestation, leaving two structures. The Eustachian valve appears at the junction of the inferior vena cava. On the other hand, the coronary valve appears at the mouth of the coronary sinus. To a high or low level, the tenacity of the right leaflet of the venous sinus valve leads to a wide range of abnormalities, which comprise of a prominent the Chiari network, Eustachian valve, and Cor triatriatum dexter CTD [3]. CTD is another rarer form of cor triatriatum. CTD can be diagnosed by echocardiography and magnetic resonance imaging [1]. In CTD, the right valve of the sinus venous endured, splitting up the right atrium into binary compartments. It is challenging to identify the distinctive features as the symptoms of CTD are similar to those of Ebstein's anomaly [6].

CTD is usually associated with right-side faults caused by abnormal fetal circulation. Among these, the most frequent is stenosis or atresia of the pulmonary valve, tricuspid valve abnormalities, and atrial septal defect [7]. Clinical signs are contingent upon the degree of obstruction in the heart, and there may be an absence of symptoms. It can contribute to trapped catheters, embolisms, or supraventricular arrhythmias [3]. The presentation of Cor triatriatum dexter differs from other heart conditions, and the development of the cardiac lesions can make the condition life-threatening [8]. In infants, the disease is characterized by heart failure and poor feeding. In adults, heart failure and stroke are presentations of CTD. According to the case report, the 40-year-old patient has been repeatedly admitted to hospitals due to his heart condition. Failure to afford healthcare impeded them from managing CTD, making the ailment grow and become chronic. Early interventions through surgery would have helped to enhance the survival rate. Patients with Cor triatriatum aged below 19 years who underwent surgery had an 83% survival rate as they received management options from cardiovascular surgeons [9]. The practitioners would help decipher the complex anatomy of the patient’s heart. The insight from the analysis will help establish the cause of the congestion, such as an inflow obstruction. Cardiac catheterization diagnosis can show if there is any pulmonary artery hypertension or pulmonary venous blocking.

The individuals had a clear case of CTD in childhood, and lack of treatment led to the isolated abnormality in adulthood. Patients like the 40-year-old suffer from reduced blood flow in the pulmonary. Any form of obstruction in the right ventricle will lead to an undesirable case of cyanosis. Transesophageal echocardiography-guided can be administered through surgery, which will help improve pulmonary blood flow and prevent stroke risks in the CTD patient. In children, the clinical manifestation of CTD depends on factors such as sinoatrial orifice size, an atrial septal defect, TV hypoplasia, and obstruction of right ventricular outflow [10]. The patient may be at risk of death since a low supply of oxygen in the body. Medication such as digoxin would not help the 40-year-old since there is limited blood flow, and it will be challenging to enhance the ventricular rate without surgery. Echocardiography and magnetic resonance imaging need to be conducted to inform the treatment plan. Alcohol is harmful to the individual and may have contributed to making the condition chronic. It is reported the 40-year-old consumes one or two beers a day. Alcohol contributes to further heart abnormalities as the heart chambers deflate, limiting normal systolic function [11]. The right atrium and ventricle will deflate unevenly, contributing to issues of life arrhythmia. The irregular heartbeats will affect the four chambers and limit the flow of oxygen, carbon dioxide, and blood in the body. In other instances, there can be inflammations. It would make the medical practitioners misdiagnose a patient and not provide a long-term solution to a patient. In addition, excessive alcohol will cause impaired contraction, making the heart less efficient. When consuming alcohol, the body produces Acetaldehyde, which is responsible for breaking down the alcohol. The toxic material can accumulate in the heart, considering low blood movement. The contraction will occur as it takes more time for the toxic chemical to break down the alcohol.

The patient is also affected by fluid buildup. When the heart is not pumping blood correctly, there will be a buildup of fluids, which is a common cause of post-discharge mortality [12]. The symptom is common in patients with long-term heart conditions such as CTD. The poor fluid retention, there are higher indications of deteriorating heart failure. The cause for the patient can be non-compliance with diet. A pericardium surrounds the heart, and any disease will lead to a buildup of fluids. The fluids in the delicate layers will strain the heart and its ability to regulate blood flow. The 40-year-old had troponin I levels at < 0.006, and the protein is responsible for muscle contraction. The high level of troponin complex is an indication of heart failure. Anatomic images show that atrial remnants reduce the ability of atrial septal defect [13]. It means that percutaneous catheterization may not be effective if a patient suffering from CTD or any heart condition does not watch their diet and prevent consumption of alcohol. High alcohol consumption leads to a buildup of calcium in the heart cells. Some of the calcium will attach to the troponin and cause a change in shape. That will expose the active sites and contribute to extreme contraction of the heart. The contraction will occur since the calcium will trigger a macular force. The troponin levels show that the patient has high levels of calcium. Regulating the complex proteins will allow an individual to watch their diet to prevent more abnormalities. Ischemic changes start to occur due to the closure of ASD, and a diagnostic dilemma can arise as percutaneous catheterization may not handle the technical changes [13]. Device selection would be fundamental to guarantee the 40-year-old patient will benefit from the surgery.

Results of the physical examination will be necessary to diagnose the patient with CTD. For instance, the rate a rhythm appears to be irregular, and there has been an increase in chest tightness. These are vital signs, and practitioners can miss some of the episodes since the symptoms occur intermittently in CTD patients. Comprehensive echocardiography is needed, and that will be helpful to exclude other complex heart diseases and have a correct diagnosis. Other conditions can present systemic venous abnormalities similar to CTD and make the patient continue to suffer. A satisfactory surgical repair, including excision of the membrane, can help reduce the obstruction caused by Cor triatriatum. Besides, a postoperative program is needed to ensure the 40-year-old will be at lost risk of encountering more complications. It should include a change in diet and a reduction in alcohol consumption. The patient would recover from the rear heart condition and enhance their survival rate.

CONCLUSION

Cor triatriatum dexter (CTD) is an uncommon heart condition affecting humans. It affects the right atrium, which will be separated into two and affect blood flow and essential materials. The clinical presentations differ by age and degree of obstruction. Its incidence is about 0.025% of all heart conditions. The condition contributes to the restricted flow of oxygenated blood due to percutaneous closure of atrial septal defect (ASD) caused by the rigidity of the atrium and centrioles. Echocardiography is essential in examining any abnormality in the right atrium, which will help diagnose CTD. The case report shows that the 40-year-old has been sick since childhood and could not access treatment due to a lack of insurance. The patient's symptoms include rate, rhythm control, anticoagulation, and thrombotic prophylaxis. Diet and high alcohol consumption could contribute to the buildup of fluids and heart failure. Comprehensive echocardiography and percutaneous catheterization can be effective in eliminating the condition. However, a treatment plan that should include a diet and alcohol reduction can help improve the patient's wellbeing in the future.

  1. Nassar PN, Hamdan RH (2011) Cor triatriatum sinistrum: Classification and imaging modalities. Eur J Cardiovasc Med 1(3): 84-87.
  2. Lee YS, Kim KS, Lee JB, Ryu JK, Choi JY, et al. (2007) Cor triatriatum dexter assessed by three‐dimensional echocardiography reconstruction in two adult patients. Echocardiography 24 (9): 991-994.
  3. López JCG, Perez IS, Cazzaniga M, de León JP, Diéguez CCG (2005) A rare form of cyansing heart disease due to tricuspid supravalvular obstruction in the infant. Revista española de cardiología 58(12): 1470-1472.
  4. Hoye DJ, Wilson EC, Fyfe DA, Guzzetta NA (2010) Cor triatriatum dexter: A rare cause of neonatal cyanosis. Anesth Analg 110(3): 716-718.
  5. Alghamdi MH (2016) Cor triatriatum dexter: A rare cause of cyanosis during neonatal period. Ann Pediatr Cardiol 9(1): 46-48.
  6. Binish A, Meredith A, Siddiqui WJ (2021) Cor Triatriatum. StatPearls [Internet]. StatPearls Publishing. Available online at: https://www.ncbi.nlm.nih.gov/books/NBK534243/
  7. Eroglu ST, Yildirir A, Simsek V, Bilgi M, Ozin B, et al. (2004) Cor triatriatum dexter, atrial septal defect, and Ebstein's anomaly in an adult given a diagnosis by transthoracic and transesophageal echocardiography: A case report. J Am Soc Echocardiogra 17(7): 780-782.
  8. Waqas U, Sattar Y, Rauf H, Roomi S, Shah MI (2019) A systematic review of a Long-forgotten cause of atrial fibrillation and stroke: Cor triatriatum. Cureus 11(12): e6371.
  9. Brotons JAS, Pardo FJL, Puras MJR, Haldon JEL, et al. (2010) Cor triatriatum dexter in adults. Revista Española de Cardiología (English Edition) 63(8): 998-999.
  10. Kalangos A, Shatelen N, Demyanchuk V, Ruban N, Sfyridis P, et al. (2020) Cor triatriatum dexter in children: Literature review and case report. JTCVS Techniques 4: 254-258.
  11. Kofler T, Wolfrum M, Kobza R, Kretschmar O, Toggweiler S, et al. (2022) An Extremely Rare Congenital Muscle Bundle Crossing the Right Atrial Cavity. Case Rep 4(3): 128-132.
  12. Barbieri F, Schröder M, Beyhoff N, Landmesser U, Reinthaler M, et al. (2021) Percutaneous Edge-to-Edge Tricuspid Valve Repair in a Patient with Cor Triatriatum Dexter: A Case Report. J Cardiovasc Dev Dis 8(9): 111.
  13. Chen PH, Yi CHL, Dai ZK, Chen ICh, Lo ShHS, et al. (2021) A Rare Complication During Transcatheter Closure of Double Atrial Septal Defects with Incomplete Cor Triatriatum Dexter: A Case Report. Front Cardiovasc Med 8: 815312.