Cor triatriatum dexter (CTD) is a rare but benign congenital heart abnormality where the right atrium is septated due to a prominent eustachian valve (EV). 1Zainudin A.R. Tiong K.G. Mokhtar S.A. Cor triatriatum dexter: A rare cause of childhood cyanosis.Ann Pediatr Cardiol. 2012 Jan; 5: 92-94Crossref PubMed Scopus (11) Google Scholar The EV is an embryologic structure that lies at the junction of the IVC and protrudes into the inferior right atrial cavity. 2Gad A. Mannan J. Chhabra M. Zhang X.X. Narula P. Hoang D. Prominent Eustachian valve in newborns: a report of four cases.AJP Rep. 2016 Mar; 6: e33-e37PubMed Google Scholar It usually regresses after birth upon closure of the foramen ovale . 2Gad A. Mannan J. Chhabra M. Zhang X.X. Narula P. Hoang D. Prominent Eustachian valve in newborns: a report of four cases.AJP Rep. 2016 Mar; 6: e33-e37PubMed Google Scholar Here we report a case of CTD managed by double balloon dilatation. Here, we report a case of CTD in a seven-year-old girl, born full-term, has had persistent cyanosis since birth. Initially diagnosed with patent foramen ovale (PFO) and a right-to-left shunt at a local facility, her condition lacked a definitive diagnosis and treatment. Due to persistent cyanosis, she was referred to our center. Upon initial examination at our hospital. During the initial examination she exhibited Cyanosis (Spo2 down to 86%) other vital signs are within normal, her weight was 16 kg, peripheral pulses were felt, and distal perfusion was normal, digital clubbing noted in the upper limb. During auscultation, the patient had normal first and second heart sounds. Apex wasn't displaced, no thrill or murmur of significance. She underwent routine labs; the results were within normal apart from elevated Hemoglobin and erythrocytosis. Echocardiography revealed the presence of CTD, attributable to a prominent EV directing the inferior vena cava (IVC) blood flow to left atrium throw atrial septal defect. This was thought to have created the veno-arterial shunt. A cardiac magnetic resonance imaging (MRI) was done to rule out extracardiac anomalies which showed normal result. After obtaining consent, patient was taken to cath lab. Under general anesthesia, the groin were prepped and dreped as well as the neck in case it was needed. Transesophageal echocardiogram (TEE), 2D showed prominent Eustachian valve, Color Doppler imaging revealed the IVC flow being guided by the Eustachian valve to the left atrium through an interatrial communication (Figure 1A). Bubble study also showed the same findings. Therefore, we proceeded for the Cadiac cath. After accessing the left Femoral vein. ,a 5.2 Fr Judkin special catheter was inserted through the left femoral vein (LFV) sheath to the IVC. IVC angiography showed the injected contrast tunneled through Atrial septal defect (ASD) to the left atrium (Figure 2A). The IVC size at the junction of RA was around 23 mm. Since CTD was fenestrated membrane, the catheter was able to advance to the right atrium (RA) then to the SVC, The CTD tunnel is typically fenestrated, allowing the catheter to pass through. If the catheter cannot pass, alternative approaches may be considered, such as accessing the lesion through the jugular vein. Another option is to use femoral vein access and break through the CTD membrane using a chronic total occlusion (CTO) or transseptal needle to proceed with the procedure, another injection was made to the superior vena cava (SVC) which showed the contrast draining to the right atrium then to right ventricle and eventually to the pulmonary artery (PA) Figure 2B.Figure 2Pre-intervention cardiac angiography. (2A) shows a still image of an injection in the IVC showing the contrast directly tunneled to left atrium to the left ventricle then to the aorta. (2B) shows the SVC injection with the contrast dye draining to the right atrium to the right ventricle, then to the pulmonary artery. (2C) shows the double ballooning technique in distortion of the eustachian valve.View Large Image Figure ViewerDownload Hi-res image Download (PPT) A decision was made to disrupt the Eustachian valve during a cardiac catheterization procedure to redirect the IVC blood flow to the correct pathway, thereby avoiding the need for major cardiac surgery. The wire was placed and parked in the innominate vein and the balloon was placed in position. The technique was accomplished using a double balloon technique. We aimed to choose a ballooning size that is close or equal to IVC size to achieve maximum benefit of the procedure. Since IVC diameter was 23 mm we elected to choose two 14 x 20 Atlas Gold balloons (C.R. Bard, Murray Hill, NJ, USA). (Figure 2C) A successful disruption of the EV membrane was accomplished and effective redirection of blood to its original pathway was achieved. Finally, angiography showed all IVC returned normally to the right side. TEE revealed a left-to-right shunt across the previously identified ASD and the blood going through normal pathway, Figure 1B. The patient's oxygen saturation immediately improved to 95%. No minor or major complications were observed during or after the intervention. The results remained consistent over the following days. Outpatient follow-up showed normal saturation, and Transthoracic Echocardiography (TTE) findings were unchanged. This case of CTD, which is caused by abnormal right sinus valve development, illustrates how the aberrant EV significantly widened the right-to-left shunt. 1Zainudin A.R. Tiong K.G. Mokhtar S.A. Cor triatriatum dexter: A rare cause of childhood cyanosis.Ann Pediatr Cardiol. 2012 Jan; 5: 92-94Crossref PubMed Scopus (11) Google Scholar A noticeable EV can be big enough to create a blockage in the flow coming in through the IVC. It may obstruct blood flow from the IVC to the right atrium if it is not dilated or has not retracted since birth. 2Gad A. Mannan J. Chhabra M. Zhang X.X. Narula P. Hoang D. Prominent Eustachian valve in newborns: a report of four cases.AJP Rep. 2016 Mar; 6: e33-e37PubMed Google Scholar Our patient's EV was clearly visible in imaging data and did not regress. She was initially diagnosed with a right-to-left shunt across the ASD at birth but was lost to follow-up until her severe cyanosis led to a referral to our institution. CTD can often be misdiagnosed due to its rarity and subtle presentation 3Jha A.K. Makhija N. Cor Triatriatum: A Review.Semin Cardiothorac Vasc Anesth. 2017 Jun; 21: 178-185Crossref PubMed Scopus (37) Google Scholar. We encourage general pediatricians and pediatric cardiologists to consider this diagnosis in cases of unexplained cyanosis and persistent right-to-left shunts. Our case highlights the value of transesophageal echocardiography (TEE) in providing crucial diagnostic information. However, despite its limitations, transthoracic echocardiography (TTE) remains the dominant diagnostic tool. 3Jha A.K. Makhija N. Cor Triatriatum: A Review.Semin Cardiothorac Vasc Anesth. 2017 Jun; 21: 178-185Crossref PubMed Scopus (37) Google Scholar,4Caridi-Scheible M. Guyton R. Richter E. Glas K. Rare congenital obstruction of the inferior vena cava diagnosed by intraoperative transesophageal echocardiography.CASE (Phila). 2020 Feb 10; 4: 139-141PubMed Google Scholar,5Zhang H. Liu W. Ma J. Liu H. Li L. Zhou J. et al.Pitfalls of using imaging technique in the presence of eustachian valve or chiari network: Effects on right-to-left shunt and related influencing factors.Diagnostics (Basel). 2022 Sep 21; 12: 2283Crossref PubMed Scopus (1) Google Scholar Additionally, this report highlights the range of options for treating CTD. We indicate that balloon dilatation may be a first-choice procedure for individuals who have no other cardiac lesion that might require surgical correction. Interventions of this kind have been documented in adult patients. 4Caridi-Scheible M. Guyton R. Richter E. Glas K. Rare congenital obstruction of the inferior vena cava diagnosed by intraoperative transesophageal echocardiography.CASE (Phila). 2020 Feb 10; 4: 139-141PubMed Google Scholar Such an intervention is commonly reported among adult patients and not commonly used in the pediatric population, although cases of CTD with ASD were successfully managed with balloon dilation and percutaneous closure of the septal defect in a 2022 report. 6Hasnie U.A. Prejean S.P. Ahmed A.N. Ahmed M.I. Law M.A. Transcatheter balloon dilatation of cor triatriatum dexter with percutaneous atrial septal defect closure.J Cardiol Cases. 2021 Jul 23; 25: 68-71Abstract Full Text Full Text PDF PubMed Scopus (1) Google Scholar In our case report, we opted for the double balloon technique primarily for its overall safety features, particularly when working near the inferior vena cava (IVC) to reduce the risk of IVC injury. While larger sheaths required for larger balloons can stress the femoral veins, the double balloon technique is distinguished by several key advantages that are beneficial in a variety of scenarios. These include enhanced control and precision during the procedure. Even though many CTD instances can be treated conservatively, if patient is asymptomatic or minimal cyanosis, or may resolve spontaneously, our patient's severe symptoms require medical attention. In contrast to conventional open-heart surgery, double balloon dilatation provided a minimally invasive method for reversing the orientation of the shunt and efficiently alleviating symptoms. 6Hasnie U.A. Prejean S.P. Ahmed A.N. Ahmed M.I. Law M.A. Transcatheter balloon dilatation of cor triatriatum dexter with percutaneous atrial septal defect closure.J Cardiol Cases. 2021 Jul 23; 25: 68-71Abstract Full Text Full Text PDF PubMed Scopus (1) Google Scholar Although surgery is typically used to treat cases like these, we provided a less intrusive option that produced comparable results. 6Hasnie U.A. Prejean S.P. Ahmed A.N. Ahmed M.I. Law M.A. Transcatheter balloon dilatation of cor triatriatum dexter with percutaneous atrial septal defect closure.J Cardiol Cases. 2021 Jul 23; 25: 68-71Abstract Full Text Full Text PDF PubMed Scopus (1) Google Scholar In summary, the successful application of double balloon dilatation in the management of CTD brought on by a substantial EV represents a noteworthy development in non-surgical intervention techniques. This case highlights how important it is to take into account this minimally invasive approach as a feasible option, especially for young patients, since it provides quick clinical improvement and shorter recovery time following the procedure. -CTD membrane can be disrupted using Double balloon technique to lessen the risk of IVC injury or lesions that are difficult to treat, and those who might need frequent vascular access, particularly among the pediatric population.-This case highlights how important it is to consider this minimally invasive approach as a feasible option, especially for young symptomatic patients, since it provides quick clinical improvement and shorter recovery time following the procedure. This study did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
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