Sinus venosus atrial septal defect with anomalous right superior pulmonary vein inflow into the vena cava superior in a middle-aged woman: a case report

    Authors

    Keywords

    atrial septal defect, congenital heart disease, echocardiography

    DOI

    https://doi.org/10.15836/ccar2021.187

    Full Text

    Introduction : Atrial septal defect (ASD) is the second most common adult congenital heart disease, usually asymptomatic until the third decade. Superior sinus venosus defect (SVASD) account for 5% of ASD and it is usually associated with the partial or complete connection of right pulmonary veins to vena cava superior (SVC) or right atrium (RA) ( 1 , 2 ). Case report : We report a case of a 45-year-old woman with previously known thyroiditis and hyperprolactinemia. She was referred for echocardiographic examination after an accidental finding of mid-systolic murmur during preoperative preparation for ovarian cyst surgery. She worked as a waiter and reported exertional dyspnea. An electrocardiogram revealed sinus rhythm with the right bundle branch block. Transthoracic echocardiography (TTE) demonstrated a normal-sized left heart with preserved systolic function (LVEF 70%), a dilated RA and right ventricle (RV) without signs of pulmonary hypertension. Cardiac magnetic resonance (CMR) showed a dilated RV (end-diastolic diameter 46 mm), dilated RA and dilated pulmonary artery (diameter 33 mm). During the CMR scan there was constantly a high concentration of contrast in the RV which raised suspicion of shunt presence. Transesophageal echocardiography using contrast revealed SVASD ( Figure 1 , Figure 2 ). The patient was referred for computer tomography angiography which demonstrated superior SVASD, 16 mm in width. The right superior pulmonary vein had abnormal inflow into SVC, while the right inferior and both left pulmonary veins had typical anatomical inflow into the left atrium. Cardiac scintigraphy with technetium-99 confirmed the existence of a left-right shunt, Qp: Qs ratio of 1.7: 1. Surgical repair was performed by forming an intraatrial patch using autologous pericardium and dilating plastic of the SVC and RA with a xenopericardial patch. Postoperative recovery went well and control TTE showed less dilated RV with good patch position and no signs of shut over intraatrial septum. Transesophageal echocardiography. Arrow pointing at a superior sinus venosus atrial septal defect. LA - left atrium; RA - right atrium; SVC - superior vena cava. Transesophageal echocardiography with contrast study. LA - left atrium; RA - right atrium; SVC - superior vena cava. Conclusion : We represented a rare case of congenital heart disease, diagnosed in a middle-aged patient. Having a patient with a dilated right heart and normal-sized left heart without signs of pulmonary hypertension needs to raise suspicion of L-R shunt existence and further investigation should be done.

    Cardiologia Croatica
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    Sinus venosus atrial septal defect with anomalous right superior pulmonary vein inflow into the vena cava superior in a middle-aged woman: a case report

    Extended Abstract
    Issue5-6
    Published
    Pages187
    PDF via DOIhttps://doi.org/10.15836/ccar2021.187
    atrial septal defect
    congenital heart disease
    echocardiography

    Authors

    Ivona Mustapić*ORCIDUniversity of Split School of Medicine, Split, Croatia
    Zora Sušilović GrabovacORCIDUniversity of Split School of Medicine, Split, Croatia
    Darija Baković KramarićORCIDUniversity of Split School of Medicine, Split, Croatia

    Full Text

    Introduction : Atrial septal defect (ASD) is the second most common adult congenital heart disease, usually asymptomatic until the third decade. Superior sinus venosus defect (SVASD) account for 5% of ASD and it is usually associated with the partial or complete connection of right pulmonary veins to vena cava superior (SVC) or right atrium (RA) ( 1 , 2 ). Case report : We report a case of a 45-year-old woman with previously known thyroiditis and hyperprolactinemia. She was referred for echocardiographic examination after an accidental finding of mid-systolic murmur during preoperative preparation for ovarian cyst surgery. She worked as a waiter and reported exertional dyspnea. An electrocardiogram revealed sinus rhythm with the right bundle branch block. Transthoracic echocardiography (TTE) demonstrated a normal-sized left heart with preserved systolic function (LVEF 70%), a dilated RA and right ventricle (RV) without signs of pulmonary hypertension. Cardiac magnetic resonance (CMR) showed a dilated RV (end-diastolic diameter 46 mm), dilated RA and dilated pulmonary artery (diameter 33 mm). During the CMR scan there was constantly a high concentration of contrast in the RV which raised suspicion of shunt presence. Transesophageal echocardiography using contrast revealed SVASD ( Figure 1 , Figure 2 ). The patient was referred for computer tomography angiography which demonstrated superior SVASD, 16 mm in width. The right superior pulmonary vein had abnormal inflow into SVC, while the right inferior and both left pulmonary veins had typical anatomical inflow into the left atrium. Cardiac scintigraphy with technetium-99 confirmed the existence of a left-right shunt, Qp: Qs ratio of 1.7: 1. Surgical repair was performed by forming an intraatrial patch using autologous pericardium and dilating plastic of the SVC and RA with a xenopericardial patch. Postoperative recovery went well and control TTE showed less dilated RV with good patch position and no signs of shut over intraatrial septum. Transesophageal echocardiography. Arrow pointing at a superior sinus venosus atrial septal defect. LA - left atrium; RA - right atrium; SVC - superior vena cava. Transesophageal echocardiography with contrast study. LA - left atrium; RA - right atrium; SVC - superior vena cava. Conclusion : We represented a rare case of congenital heart disease, diagnosed in a middle-aged patient. Having a patient with a dilated right heart and normal-sized left heart without signs of pulmonary hypertension needs to raise suspicion of L-R shunt existence and further investigation should be done.