|Year : 2016 | Volume
| Issue : 2 | Page : 119-121
Cor triatriatum dexter variant identified by critical congenital heart disease screening
Yonit Lax1, Christine Walsh2, Kenan Stern2, M Susan Latuga3
1 Department of Pediatrics, The Children's Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, New York 10461-2301, USA
2 Department of Pediatrics, Division of Cardiology, Albert Einstein College of Medicine, Bronx, New York 10461-2301, USA
3 Department of Pediatrics, Division of Neonatology, Albert Einstein College of Medicine, Bronx, New York 10461-2301, USA
|Date of Web Publication||8-Apr-2016|
M Susan Latuga
Department of Pediatrics, Division of Neonatology, Albert Einstein College of Medicine, 1825, Eastchester Road, Suite 725, Bronx, New York 10461-2301
Source of Support: None, Conflict of Interest: None
Critical congenital heart disease (CCHD) screening utilizes pulse oximetry to detect heart disease in the neonatal period. While the primary targets for screening are common cyanotic lesions, screening may result in a diagnosis of rare cyanotic lesions such as cor triatriatum dexter (CTD). CTD is a rare congenital cardiac malformation caused by persistence of the right valve of the sinus venosus, which divides the right atrium into two chambers. Here, we describe an asymptomatic female newborn with a positive screening for CCHD diagnosed with a variant of CTD. To the best of our knowledge, this is the first report of CTD diagnosed after a positive CCHD screen. With the advent of CCHD screening, identification of rare congenital heart diseases in asymptomatic infants may increase. CCHD screening allowed diagnosis and management without surgical repair of CTD in a clinically asymptomatic infant.
Keywords: Congenital heart disease, cor triatriatum dexter, pulse oximetry, screening
|How to cite this article:|
Lax Y, Walsh C, Stern K, Latuga M S. Cor triatriatum dexter variant identified by critical congenital heart disease screening. J Clin Neonatol 2016;5:119-21
|How to cite this URL:|
Lax Y, Walsh C, Stern K, Latuga M S. Cor triatriatum dexter variant identified by critical congenital heart disease screening. J Clin Neonatol [serial online] 2016 [cited 2021 Apr 12];5:119-21. Available from: https://www.jcnonweb.com/text.asp?2016/5/2/119/179923
| Introduction|| |
Despite varying definitions in the literature, critical congenital heart disease (CCHD) can cause life-threatening complications or death without intervention.  Delayed diagnosis of CCHD has been associated with adverse neurodevelopmental outcomes, cardiac arrest, and death.  Because hypoxia with most CCHD may not be clinically detected with visible cyanosis, pulse oximetry has been utilized to screen for CCHD in the neonatal period. While the primary targets for screening are common cyanotic lesions, screening may result in the diagnosis of rare cyanotic lesions such as cor triatriatum dexter (CTD). To the best of our knowledge, this is the first report of CTD diagnosed after a positive CCHD screen.
| Case Report|| |
A full-term female infant was delivered via spontaneous vaginal delivery to a gravida two, para one mother, with an uncomplicated medical and pregnancy history. Due to meconium-stained amniotic fluid, she was intubated with no meconium noted below the vocal cords. By 1 min of life, the patient had an APGAR of nine and was transferred to the newborn nursery. The patient tolerated ad-lib feeding and was otherwise asymptomatic. On the day of life two, a screen for CCHD revealed preductal (right upper extremity) and postductal (right lower extremity) transcutaneous oxygen saturations ranging from the mid-70s to low-80s without a differential diagnosis. The patient was transferred to the Neonatal Intensive Care Unit (NICU) for further evaluation.
In the NICU, an electrocardiogram and four limb blood pressures were normal. The patient was placed on continuous positive airway pressure (CPAP) with supplemental oxygen titrated to 100% without an increase in oxygen saturations. A chest X-ray showed a normal cardiothymic silhouette and no pulmonary findings. An echocardiogram showed a large, redundant membrane arising from the anterior aspect of the entrance of the inferior vena cava to the right atrium and prolapsing into the tricuspid valve during diastole, resulting in a narrow effective right ventricular inflow. There was right to left flow through a patent foramen ovale. Tricuspid valve morphology was normal as was right ventricular size [Figure 1].
|Figure 1: Echocardiography showed membrane in right atrium. (a) A subcostal short-axis view demonstrates the membrane (arrow) attaching to the anterior aspect of the entrance of the inferior vena cava to the right atrium. (b) On an apical color-compare view, the membrane prolapses through the tricuspid valve annuals in diastole. A narrow jet of tricuspid inflow enters the right ventricle around the anterolateral aspect of the memberane|
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On the day of life four, the patient was weaned off of CPAP and onto room air. Throughout her admission, the patient tolerated ad lib feeds well with adequate weight gain and no breathing difficulty, cyanosis, or diaphoresis. The echocardiogram done prior to discharge on the day of life six was unchanged. At the time of discharge, oxygen saturations improved to the low 90s.
On outpatient follow-up, a family history of neurofibromatosis was noted and six café au lait spots were found on examination. A geneticist confirmed the diagnosis of neurofibromatosis type 1. At the time of this report, plan of care involves careful observation without surgical intervention. The patient's last pulse oximetry reading was 100% at 1-year of life.
| Discussion|| |
In 2011, the United States Health and Human Services Secretary recommended that CCHD screening can be included in the uniform screening panel. While the American Academy of Pediatrics and the American Heart Association have endorsed CCHD screening, individual states must opt to include CCHD screening as a mandate for all infants.
In June 2013, New York State adopted a standardized approach toward CCHD screening: Measurement of preductal (right hand) and postductal (either foot) oxygen saturation at 24-48 h of age. A positive screen is any measurement <90% or a >3% difference between pre- and post-ductal saturations for three separate measurements occurring at least 1 h apart from one another.
CTD has an incidence of <1% of all congenital heart disease. In the human fetus, the right sinus venosus valve typically regresses between 9 and 15 weeks gestation, leaving behind the Eustachian valve More Details and crista terminalis as remnants. CTD results from failed regression of the right sinus venosus valve with varying degrees of the obstruction of systemic venous return into the right ventricle. This blood flow is redirected across the patent foramen ovale causing clinical cyanosis.
The clinical severity of CTD depends on the degree of resorption of the right sinus venosus valve.  In classic CTD, the sinus venosus valve extends all the way to the crista terminalis, septating the right atrium into an inflow portion that receives the superior and inferior vena cava, and a distal chamber that consists of the right atrial appendage and tricuspid valve orifice. Small defects in this membrane usually permit a small amount of right ventricular inflow and the majority of caval return is redirected across the foramen ovale. Our patient exhibited a prominent remnant of the sinus venosus valve that was large, mobile, redundant, and not fenestrated. It obstructed inflow into the tricuspid valve by prolapsing into the right ventricle during diastole.
Clinically, CTD has a range of presentations. On postmortem autopsy after termination, CTD has been found in association with severe right ventricular hypoplasia and hydrops fetalis. , During the neonatal period, CTD may present with cyanosis and respiratory distress. , CTD has been found with a variety of other congenital heart diseases including pulmonary artery stenosis and tricuspid valve atresia.  CTD has also been associated with omphalocele and neurofibromatosis. ,
Surgical resection or a percutaneous transluminal approach is indicated when symptoms of obstruction to flow or persistent desaturation are present.  In this patient, the echocardiogram raised the possibility that the membrane might prolapse into the tricuspid valve and obstruct flow [Figure 1]. Over time, oxygen saturations improved with no signs or symptoms of obstruction. It was, therefore, elected to observe the infant closely as an outpatient with pulse oximetry and echocardiography. Of concern, is the continued right-to-left flow across the patent foramen ovale, which could potentiate a paradoxical embolus despite the flow being minimal.
In the reported cases, CTD has been diagnosed in symptomatic infants with clinical evidence of obstruction. With the advent of CCHD screening, identification of rare congenital heart diseases in asymptomatic infants may increase. Existing paradigms for surgical and medical management may need to be adjusted in clinically well infants with congenital heart disease. In summary, CCHD screening allowed diagnosis and management without surgical repair of CTD, in a clinically asymptomatic infant.
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Conflicts of interest
There are no conflicts of interest.
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