ivemark syndrome is a heterotaxy syndrome affecting multiple organs of the body , often presenting with congenital asplenia and cardiac problems [ 1 , 2 ] . commonly seen as right isomerism , typically there are two right - sided lungs and right - sided atria . the heart contains septal defects with right ventricular hyperplasia . additionally , the pulmonary venous return is irregular , long , and tortuous as it winds back to the heart . life expectancy is heavily reduced with 10% of cases dying within the first 24 h and 80% dying within the first year of life . these cardiac problems may include obstruction of pulmonary venous connections , seen in 48% of patients with heterotaxia and total anomalous pulmonary venous return ( tapvr ) . here , we discuss an unusual case of tapvr causing bronchial stenosis using cardiac ct angiogram to produce 3d functional figures displaying the anomalous pulmonary venous back to the left atrium . a 3-month - year - old male with total anomalous pulmonary venous connection , a double - outlet right ventricle , congenital stenosis of the pulmonary artery valve and heterotaxy syndrome with asplenia is one of twins who presented clinically stable . his right - sided pulmonary veins use an alternate route back to the heart as they drain into a right - sided vertical vein which passes under the carina and then back over the left main bronchus , joining the left lower lobe pulmonary vein to empty into the left atrium ( figs 1 and 2 ) . as a result , also , there is systemic venous communication with the left - sided vertical vein , left subclavian vein and the hemiazygos system ( figs 5 and 6 ) . the left venous vertical vein is shown passing over the left mainstem bronchus ( yellow ) as it enters the heart . multiple pulmonary vein branches are shown conjoining together to form the left - sided vertical vein ( white arrow ) . it dips under the carina and loops back over the left mainstem bronchus ( yellow ) . the left venous vertical vein is shown passing over the left mainstem bronchus ( yellow ) as it enters the heart . multiple pulmonary vein branches are shown conjoining together to form the left - sided vertical vein ( white arrow ) . it dips under the carina and loops back over the left mainstem bronchus ( yellow ) . additionally , there is a clockwise rotation of the aortic root , but the coronary arteries still originate from their respective sinuses ( figs 3 and 4 ) . there is no aortic insufficiency or stenosis , nor is there any aortic coarctation ( figs 5 and 6 ) . he also has both a large ventricular septal defect ( vsd ) with a septum primum atrial septal defect ( asd ) , with a double - outlet right ventricle and left ventricle hypoplasia ( figs 5 and 6 ) . figure 3:ct 3d showing anterior pulmonary arterial circulation ( blue ) . both left and right branches are nearly identical , characterized by the two right - sided lungs . the left - sided subclavian vein ( light blue ) brings blood to the right atrium ( light blue ) . the right ventricle ( dark purple ) provides outflow to both the aorta ( red ) and the pulmonary arteries ( dark blue ) . the multiple branches of the pulmonary veins ( pink ) can be seen coming together and wrapping over the left mainstem bronchus . the hypoplastic left ventricle ( white arrow ) can be seen small and shrunken slightly to the left of the large right atrium . this view focuses primarily on the anomalous branching and route of the pulmonary venous flow in relation to other vessels and airways nearby . the left - sided vertical vein can be seen traveling over the left bronchus ( white arrow ) . both left and right branches are nearly identical , characterized by the two right - sided lungs . ct 3d showing anterior heart and circulation . the left - sided subclavian vein ( light blue ) brings blood to the right atrium ( light blue ) . the right ventricle ( dark purple ) provides outflow to both the aorta ( red ) and the pulmonary arteries ( dark blue ) . the multiple branches of the pulmonary veins ( pink ) can be seen coming together and wrapping over the left mainstem bronchus . the hypoplastic left ventricle ( white arrow ) can be seen small and shrunken slightly to the left of the large right atrium . this view focuses primarily on the anomalous branching and route of the pulmonary venous flow in relation to other vessels and airways nearby . the left - sided vertical vein can be seen traveling over the left bronchus ( white arrow ) . ivemark syndrome is a unique form of heterotaxy , taking the right - sided form and thus resulting in no spleen development . many times it is associated with anomalous pulmonary venous return , with the newly oxygenated blood traveling a long or ambiguous route back to the heart . unfortunately , this kind of anomalous pulmonary venous return is different patient - to - patient contributing to the need for cardiac ct angiogram and subsequent 3d functional figures . the concurrent septum primum asd with corresponding vsd makes this issue very difficult to correct . due to the double - outlet right ventricle , there is a largely hypoplastic left ventricle and hyperplastic right ventricle . both the pulmonary and systemic circulation falls upon the pumping action of the right ventricle , when typically the left ventricle handles the systemic circulation and the right ventricle handles the pulmonary circulation . subsequently , the disuse of the left ventricle puts additional pressure on the right , increasing the pressure at which the right ventricle pumps out blood . typically , the pulmonary circulation has lower blood pressure due to the nature of the small capillaries the blood passes through . the left ventricle usually pumps harder in order to provide circulation throughout the entire length of the body . with the right ventricle now serving as pumps for both types of circulation , there is the possibility of pulmonary edema and poor blood perfusion throughout the body . without efficient oxygenation of all of the blood and the adequate circulation of oxygenated blood out of the heart , the patient will likely suffer from chronic poor perfusion and shortness of breath , as well . additionally , the pulmonary veins curve over the left mainstem bronchus causing it to narrow compared to the right mainstem bronchus . with the heterotaxy seen in this patient , both lungs are right - sided causing the same slight angle of entry into the lungs by both mainstem bronchi . the slight narrowing of the left mainstem bronchus could lead to problems with breathing and physical activity . as of yet , there have not been many attempts in permanently correcting these issues in ivemark patients . apart from a bidirectional glenn operation to shunt blood from the vena cava to the pulmonary arteries and therefore bypass the right side of the heart , these patients must be kept under close observation for visible distress due to the heart condition .