Cyanosis in patients with atrial septal defect due to systemic venous drainage into the left atrium

Maillis, Maxwell S.; Cheng, Tsung O.; Meyer, Jerry F.; Crawley, I.Sylvia; Lindsay, Joseph

doi:10.1016/0002-9149(74)90261-6

Cited by 19 publications

(4 citation statements)

References 10 publications

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“…TASVD is a rare type of congenital heart malformation. TASVD drainage requires the presence of a left-to-right shunt (PDA, ASD, or VSD) to allow the systemic venous return to reach the pulmonary circulation ( 3 ). Functional drainage of systemic venous blood into the left atrium across an atrial septal defect has been described.…”

Section: Discussionmentioning

confidence: 99%

Multimodal Imaging for Total Anomalous Systemic Venous Drainage Diagnosis and Preoperative Planning: A Case Report and Literature Review

Ding

Zhang

Sun

et al. 2022

Front. Cardiovasc. Med.

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Total anomalous systemic venous drainage (TASVD) is a rare congenital heart malformation. Here, we report a case of a 40-year-old male patient who had a total anomalous systemic venous drainage. It was diagnosed as the TASVD for the first time through multimodal imaging combined Transthoracic (TTE), transesophageal (TEE) and three-dimensional (3D-TTE) echocardiography, contrast echocardiography and computed tomography angiography (CTA). We review 15 published reports on TASVD and summarize the ultrasonographic characteristics. After intracardiac repair through ectopic venous drainage in cardiac surgery, the patient's cyanosis symptoms were alleviated greatly. Echocardiography was the first-line examination for TASVD. Multimodal imaging combined TTE, TEE, 3D TEE, contrast echocardiography and CTA was necessary for confirmed diagnosis of TASVCD.

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Section: Discussionmentioning

confidence: 99%

Multimodal Imaging for Total Anomalous Systemic Venous Drainage Diagnosis and Preoperative Planning: A Case Report and Literature Review

Ding

Zhang

Sun

et al. 2022

Front. Cardiovasc. Med.

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“…[1][2][3] This has been described as early as in 1976. 10 The very first description of atrial septal defects with cyanosis dates from 1949, where Selzer and Lewis postulate functional drainage of systemic venous blood into the left atrium. 11 These assumptions were based on postmortem examinations.…”

Section: Discussionmentioning

confidence: 99%

Cyanosis due to an isolated atrial septal defect: case report and review of the literature

2020

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Isolated atrial defects usually lead to left-to-right shunt and right ventricular volume load. Descriptions of cyanosis with this congenital heart defect are rare. We describe a rare case of inferior caval vein flow directed through an atrial septal defect in the fossa ovalis leading to severe cyanosis, but without any additional intracardiac anatomical abnormalities. The baby with clinical features of Marfan’s syndrome had an eventration of the right-sided diaphragm. Surgical closure of the defect resolved the cyanosis, but the child died 10 weeks later of severe valvar dysfunction, related to Marfan’s syndrome. Mechanisms of cyanosis in patients with atrial septal defects are discussed. Echocardiographic bubble studies both from the lower and upper half of the body may help to clarify the mechanism of an otherwise unexplained cyanosis.

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“…Previous report also showed that approximately 50% of the dogs and cats with ASD processed other congenital heart diseases including valvular defects (Chetboul et al, 2018). In terms of clinical presentation, left to right shunt of ASD can lead to right-sided volume overload and right to left shunt of ASD can cause a reduction of oxygenated blood leading to polycythemia (Maillis et al, 1974;Ikäheimo et al, 1983;Le Gloan et al, 2018). Thus, the clinical signs of PS dogs with ASD may be more severe.…”

Section: Chapter V Discussionmentioning

confidence: 95%

“…Decreased partial pressure of oxygen (PO 2 ) stimulated erythropoietin (EPO) production from the kidney and sequentially increased RBC production (Broberg et al, 2006). Many types of congenital defects such as ventricular septal defect (VSD), ASD and tetralogy of Fallot can cause secondary erythrocytosis of cyanotic congenital heart diseases (CCHD) (Maillis et al, 1974;Ikäheimo et al, 1983;Thomson et al, 1999;Gunduz et al, 2014).…”

Section: Chapter V Discussionmentioning

confidence: 99%

The left ventricular functions and heart rate variability in dogs with pulmonic valvular stenosis

Trikhun¹

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Pulmonic valvular stenosis (PS) is one of the most common congenital heart diseases in dogs that can lead to the cardiac maladaptation. Also, PS can affect the cardiac autonomic nervous system (ANS). However, clinical information regarding to systolic function and cardiac ANS alteration in PS dogs had not been fully elucidated. The objectives of this study were to evaluate cardiac electrical property, left ventricular (LV) function, and to assess cardiac ANS from heart rate variability (HRV) analysis in PS dogs compared with healthy dogs. The dogs in this study were divided into 2 groups, PS dogs (n=13) and healthy control dogs (CONT) (n=12). Physical examination, oscillometric blood pressure measurement and assessments of cardiac function including electrocardiography (ECG), echocardiography and 30-min heart rate variability (HRV) were performed. The blood sample were collected for determination of complete blood count and blood chemistries. The results showed that all PS dogs had exercise intolerance while 6 developed syncope. ECG findings indicated that PS dogs had higher amplitudes of P, and T waves (P<0.01), deeper S wave, lower R:S ratio (P<0.001) with longer QRS duration than CONT dogs (P<0.001). For cardiac structural and functional studies, the pulmonic flow velocity (PV) and pressure gradient (PG) between the right ventricle (RV) and the pulmonary artery (PA) of PS dogs were significantly higher than CONT dogs (P<0.001). PS dogs had thicker RV free wall (P<0.001) and interventricular septum (IVS) (P<0.01), bigger right atrium (RA) (P<0.001) with smaller LV chamber during diastole and systole compared with CONT dogs (P<0.001). The RV systolic function in PS dogs showed higher pulmonic valve velocity time integral (PVVTI) value (P<0.001) and longer pulmonic valve ejection time (PVET) (P<0.01). However, there was no significantly difference in HRV parameters between groups. PG had positive correlations with amplitudes of P (r=0.597, P<0.01), S (r=0.569, P<0.01) and T waves (r=0.423, P<0.05) and negative correlations with amplitude of R wave (r=-0.599, P<0.01), R:S ratio (r=-0.677, P<0.001) and QRS duration (r=-0.423, P<0.05). Also, PG had positive correlations with IVS during diastole normalized by body weight (IVSdN) (r=0.560, P<0.01), IVS during systole normalized by body weight (IVSsN) (r=0.538, P<0.01), PVVTI (r=0.812, P<0.001), PVET (r=0.408, P<0.05), right ventricular free wall thickness to left ventricular posterior wall thickness (RVFW:LVPW) ratio (r=0.688, P<0.001) and right atrium to left atrium diameter (RA:LA) ratio (r=0.802, P<0.001). In conclusion, RV hypertrophy induced by PS may lead to reduction of LV preload and function. The increased PVET in PS dogs may be one of the cardiac compensations to maintain pulmonary blood flow. However, the unremarkable cardiac ANS changes in PS dogs may not play a crucial role in chronic pathophysiological alteration of the hemodynamics.

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Cyanosis in patients with atrial septal defect due to systemic venous drainage into the left atrium

Cited by 19 publications

References 10 publications

Multimodal Imaging for Total Anomalous Systemic Venous Drainage Diagnosis and Preoperative Planning: A Case Report and Literature Review

Multimodal Imaging for Total Anomalous Systemic Venous Drainage Diagnosis and Preoperative Planning: A Case Report and Literature Review

Cyanosis due to an isolated atrial septal defect: case report and review of the literature

The left ventricular functions and heart rate variability in dogs with pulmonic valvular stenosis

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