Pulmonary Veins in Congenital Heart Disease in the Adult

Radiology ◽  
1961 ◽  
Vol 76 (6) ◽  
pp. 885-893 ◽  
Author(s):  
Robert S. Ormond ◽  
Andrew K. Poznanski ◽  
Arch W. Templeton
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Pulmonary Veins

scholarly journals Contemporary use of Selexipag in pulmonary arterial hypertension associated with congenital heart disease: a case series

2020 ◽  
Author(s):  
Sarah Blissett ◽  
David Blusztein ◽  
Vaikom S Mahadevan
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Side Effects ◽  
Arterial Hypertension ◽  
Exercise Capacity ◽  
Congenital Heart ◽  
Pulmonary Veins ◽  
Case Series ◽  
Pulmonary Arterial

Abstract Background There are significant risks of parenteral prostacyclin use in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), which may limit their use. Selexipag is an oral, selective prostacyclin analogue that has been shown to reduce disease progression and improve exercise capacity in patients with PAH-CHD. Administering Selexipag in patients with PAH-CHD could potentially overcome some of the risks of parenteral therapy while improving clinical outcomes. Case summary We report five cases highlighting the clinical uses of Selexipag in patients with PAH-CHD. In the first two cases, Selexipag was initiated as part of a Treat-to-close strategy. In the third case, initiation of Selexipag improved symptoms and objective exercise capacity in a patient with Eisenmenger syndrome. In the fourth and fifth cases, rapid cross-titration protocols were used to transition from parenteral prostacyclins to Selexipag. In the fourth case, Selexipag was initiated in the context of significant side effects limiting parenteral prostacyclin use. In the fifth case, Selexipag was used to down-titrate from parenteral prostacyclins following closure of a sinus venosus atrial septal defect and redirection of anomalous pulmonary veins. Discussion Selexipag is a promising oral therapy for patients with at various stages of the spectrum of PAH-CHD to improve symptoms, exercise capacity and, in some cases, haemodynamics. Our cases also highlight practical aspects of Selexipag use including targeting the individualized maximally tolerated dose for each patient, managing side effects and managing dose interruptions.

237: CONGENITAL HEART DISEASE AND ALVEOLAR CAPILLARY DYSPLASIA WITH MISALIGNMENT OF PULMONARY VEINS

2018 ◽  
Vol 46 (1) ◽  
pp. 101-101
Author(s):  
Katharine Robb ◽  
Sook-kyung Kwon ◽  
Veerajalandhar Allareddy ◽  
Aditya Badheka
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Pulmonary Veins ◽  
Alveolar Capillary Dysplasia ◽  
Alveolar Capillary

The role of echocardiography in adult congenital heart disease

2021 ◽  
pp. 783-807
Author(s):  
Lindsay A. Smith ◽  
Mark K. Friedberg ◽  
Luc Mertens
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Adult Congenital Heart Disease ◽  
Pulmonary Veins ◽  
Diagnostic Technique ◽  
Strain Rate Imaging ◽  
Strain And Strain Rate ◽  
Adult Congenital ◽  
Dimensional Echocardiography

Echocardiography plays a key role in the diagnosis and management of adult patients with congenital heart disease and is considered the first-line diagnostic technique. Apart from traditional cross-sectional imaging, three-dimensional echocardiography and strain and strain rate imaging were introduced for specific indications and for better describing anatomical details and functional consequences of the different congenital lesions. For specific indications, additional imaging may be required including cardiac magnetic resonance imaging and computational tomography. Especially in adult congenital heart disease, echocardiographic imaging may be limited by poor acoustic windows and additional evaluation using other imaging modalities may be required. Additionally, MRI and CT imaging have proven to be extremely valuable for evaluation of right ventricular size and function and for describing extracardiac anatomy (pulmonary arteries, pulmonary veins, and aorta).

scholarly journals Prevalence of arrhythmias on the long term of adult patients with partial anomalous pulmonary venous connection treated surgically and conservatively: data from the SACHER registry and a French center

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
M Touray ◽  
J Bouchardy ◽  
M Ladouceur ◽  
M Schwerzmann ◽  
M Greutmann ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Late Complication ◽  
Pulmonary Veins ◽  
Pacemaker Implantation ◽  
Funding Source ◽  
Cardiac Symptoms ◽  
Systemic Vein ◽  
Anomalous Pulmonary Venous Connection

Abstract Introduction Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital heart disease, characterized by one or some but not all pulmonary veins anomalously connected to the right atrium or a systemic vein. PAPVC is either an isolated shunt lesion or associated with an atrial septal defect (ASD). This study compares the arrhythmic outcome of patients treated surgically with those clinically monitored. Method Clinical, surgical, imaging and invasive data of PAPVC patients were retrospectively reviewed from 7 centers from the Swiss Adult Congenital HEart disease Registry (SACHER) and one French center. Results A total of 168 patients with PAPVC were identified. Most (77%) patients underwent surgery, while the remaining (23%) ones were treated conservatively with clinical monitoring. The operated group (OG) had a significantly higher number of associated ASD (N=106, 82%) (p<0.001) and a higher number of anomalous pulmonary veins leading to a mean Qp:Qs at 2.5±1.2 before surgery (table). Moreover, the majority of patients in the OG had cardiac symptoms (N=78, 60%) and were diagnosed at a significantly younger age (table). Mean age at operation was 27±20 years. Age did not differ at latest follow-up (table). Holter recordings revealed a higher prevalence of arrhythmia in the OG (p=0.031), mainly of supraventricular tachyarrhythmias (table). The occurrence of ventricular non-sustained tachycardia and of bradyarrhythmia did not statistically differ between groups. Patients in the OG required more often medical treatment for arrhythmias: 12 (9%) needed electrophysiological study in the OG and none in the NOG (p=0.057). The amount of patients requiring a pacemaker implantation in the OG (11%) was significantly higher than that of NOG (0%) (p=0.039). Conclusion Patients after PAPVC repair present with a significant higher burden of arrhythmia than conservatively treated patients, either due to a larger shunt pre-operatively and/or as a late complication of the corrective surgery itself. Funding Acknowledgement Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): Unrestricted grant from Actelion, Switzerland

scholarly journals P648 Right heart volume overload after surgical correction of atrial septal defect sinus venosus type with partial anomalous pulmonary veins drainage

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
I Ruiz-Zamora ◽  
L Alvarez-Roy ◽  
G Pinillos-Francia ◽  
A Gutierrez-Fernandez ◽  
M Gomez-Llorente ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Vein ◽  
Congenital Heart ◽  
Pulmonary Veins ◽  
Venous Drainage ◽  
Sinus Venosus ◽  
The Right

Abstract 40 year-old male with history of congenital heart disease. Atrial septal defect (ASD) sinus venosus type associated with partial anomalous pulmonary venous drainage (PAPVD) was diagnosed during childhood and surgically repaired at the age of three. Since then the patient was asymptomatic and he was lost to follow up when reached adulthood. A transthorathic echocardiogram was performed during a hospitalization because of a complicated pneumonia. A severe dilatation of right chambers and main pulmonary artery was observed. The estimated Qp:Qs by this technique was 2.0 but the atrial septum seemed to be intact. Agitated saline was administered in this procedure and no passage of microbubbles was observed. A cardiac MRI was performed because of suspicion of anomalous pulmonary venous drainage. The findings observed in the echocardiogram were confirmed and an uncommon PAPVD was demonstrated by this technique: the superior left pulmonary vein drainaged into a dilated innominated vein. Also, a small pulmonary vein from the right upper lobe emptied into a "venous conduct" (yellow circle) located posterior to the superior cava vein (SCV) that later drainaged into the right atrium under the mouth of the SVC, posterior to the interatrial septum. Surgically correctioin of the PAPVD was performed. Discussion PAPVD is a congenital heart disease characterized by the drainage of one or several pulmonary veins –but not all of them- into the right atrium or systemic veins, which leads to left-to-right shunting. The estimated incidence of this disease ranges between 0.1 and 0.2% of the general population. The right superior pulmonary vein is the most frequently involved and is commonly associated to ASD sinus venosus type. Bilateral anomalous drainage, as occurred in this case, is exceptional. Furhermore, this case highlights the importance of long-term follow-up in patients with congenital heart disease due to the fact that, even when the disease is considered to be cured, long term complications could appear compromising the prognosis of the patient. Abstract P648 Figure. Unusual PAPVD

scholarly journals Clinical scope of early fetal echocardiography

2021 ◽  
Author(s):  
Ximena Carolina Romero Infante ◽  
Arturo Montaño Mendoza ◽  
Diana Sarmiento ◽  
María Uriel Calvo ◽  
José De la Hoz Valle
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Fetal Echocardiography ◽  
Meta Analysis ◽  
Pulmonary Veins ◽  
First Trimester ◽  
Second Trimester ◽  
Congenital Heart Malformations ◽  
Cardiac Evaluation

The chances of detecting congenital heart disease are improved following structured protocols. Fetal heart disease is one of the main serious congenital malformations. The objective of this review is to present the benefits of sequential and routine cardiac evaluation in the first and early second trimesters and to identify the structures and pathologies that can be detected at this gestational age. The databases of PubMed, Medline, MD consult, Embase, Clinical Key, Scielo, and ScienceDirect, as well as specialized texts in Spanish and English were searched for diagnostic studies, systematic reviews, and meta-analysis related to the terms “early fetal echocardiography” and “congenital heart malformations” published between 2000 and 2019. Technological advances have revolutionized the fetal echocardiographic examination making possible the diagnosis of congenital heart disease from the first and early second trimester of pregnancy. However, it should be recognized that early fetal echocardiography has limitations such as the evaluation of pulmonary veins and cardiac lesions that are progressive. The benefit found was earlier detection of pathologies with high sensitivity and specificity. A fetal cardiac evaluation sequence was included in these early fetal echocardiographic examinations due to the awareness of fetal anatomical and hemodynamic differences in the first trimester and the beginning of the second trimester. Early fetal echocardiography is a very useful tool for the detection of congenital heart disease; it allows parents to be informed about the prognosis and possible treatments early. Fetal cardiac examination in the first trimester is safe and can detect the most of heart congenital malformation.

Common Pulmonary Vein Atresia: The Role of Extracorporeal Membrane Oxygenation

PEDIATRICS ◽  
1993 ◽  
Vol 91 (2) ◽  
pp. 403-410
Author(s):  
Golde G. Dudell ◽  
Marva L. Evans ◽  
Henry F. Krous ◽  
Robert L. Spicer ◽  
John J. Lamberti
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Extracorporeal Membrane Oxygenation ◽  
Pulmonary Vein ◽  
Congenital Heart ◽  
Pulmonary Veins ◽  
Cardiac Anomaly ◽  
Membrane Oxygenation ◽  
Parenchymal Disease ◽  
Common Pulmonary Vein

Common pulmonary vein atresia is a rare form of cyanotic congenital heart disease in which the pulmonary veins join to form a blind confluence that does not communicate with the heart or the major systemic veins. Twenty-one cases have been reported since the lesion was first described in 1962; only two patients with this lesion have survived. Over a 4-year period, common pulmonary vein atresia was diagnosed in five newborns referred to the San Diego Regional Extracorporeal Membrane Oxygenation Program. All five improved dramatically as a result of venoarterial bypass. Congenital heart disease was diagnosed at autopsy in the initial case and by cardiac ultrasound and/or catheterization in the others. Surgical repair was attempted in three neonates; all three required continued extracorporeal membrane oxygenation support postoperatively because of pulmonary hypertension and severe pulmonary parenchymal disease. One infant died of respiratory insufficiency at 3 months of age. The other two survived and were discharged from the hospital. The diagnostic and therapeutic dilemmas posed by this lesion and the life-saving potential for extracorporeal membrane oxygenation in this rapidly fatal cardiac anomaly are the bases of this report.

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