Search Our Scientific Publications & Authors

Publications
find publications by category +
Translate page:

The outcome of patients with right atrial isomerism is poor.

Authors:
Marianne P Eronen Kristiina A U Aittomäki Eero O Kajantie Heikki I Sairanen Erkki J Pesonen

Pediatr Cardiol 2013 Feb 12;34(2):302-7. Epub 2012 Aug 12.

Department of Health, The Social Insurance Institution of Finland, Hoylaamontie 1 aB PL 78, 00380 Helsinki, Finland.

Right-atrial isomerism (RAI) is a heterotaxy syndrome with disturbances of left-right axis development resulting in complex heart malformations and anomalies of the thoracic and abdominal organs. To study the outcome of RAI, all data from patients diagnosed with this syndrome at Helsinki University Hospital between January 1976 and December of 2010 were reviewed. The outcomes were studied for 32 patients (38 % girls). The overall survival was 22 % at a median follow-up time of 13.8 years (range 0.1-33). Extracardiac malformations, mostly asplenic, occurred in 91 % of patients. Cardiac defects included dextrocardia in 44 % and common atrioventricular valve in 100 % of patients. Ventriculoarterial discordance or double-outlet ventricle was seen in 56 and 44 % of patients, respectively. Total anomalous pulmonary venous drainage occurred in 75 % and partially anomalous venous drainage in 13 % of patients. Pulmonary outflow-tract obstruction was identified in 91 % of patients. Cardiac arrhythmias were noted in nine patients (28 %), two of them with atrioventricular block. Cardiovascular surgery was performed in 71 % patients (N = 25), seven patients were inoperable. Biventricular repair was not possible in any of the patients. During long-term follow-up there was no significant difference between the patients with total, normal, or partially anomalous pulmonary venous drainage (P = 0.5). In conclusion, RAI is one of the most severe forms of congenital cardiac diseases. The prognosis remains poor despite modern surgical techniques. When RAI is identified during pregnancy, prenatal counseling, termination, or planning for prompt cardiac treatment after the birth is necessary.

Download full-text PDF

 Source
http://dx.doi.org/10.1007/s00246-012-0445-yDOI Listing
February 2013

Publication Analysis

Top Keywords

venous drainage
12
patients
12
patients total
8
partially anomalous
8
anomalous pulmonary
8
pulmonary venous
8
patients cardiac
8
total anomalous
4
drainage occurred
4
occurred partially
4
treatment birth
4
anomalous venous
4
ventricle patients
4
patients pulmonary
4
pulmonary outflow-tract
4
outflow-tract obstruction
4
obstruction identified
4
identified patients
4
cardiac arrhythmias
4
drainage patients
4

Keyword Occurance

Similar Publications

Postmortem Retrograde Contrasted Infusion in Thoracic Duct Outflow: Imaging Effectiveness Analysis.

Authors:
Rogério Rodrigo Ramos Mariane Gabriela Terezani Elís Claudia Ribeiro Cantarella Jose Maria Pereira de Godoy Fernando Batigalia Luciana Estevam Simonato Wagner Rafael da Silva José Martins Pinto Neto André Wilian Lozano Nilton Cesar Pezati Boer

Cureus 2022 Apr 17;14(4):e24224. Epub 2022 Apr 17.

Department of Health Sciences, Brasil University, Fernandópolis, BRA.

Background Thoracic duct (TD) anomaly can be quite variable and dangerous in surgical interventions in the neck region as there are numerous variations in its formation and topography. This highlights the importance of full knowledge about the TD and its anatomical variations. Thus, it is important to emphasize that the lack of anatomical-clinical knowledge or surgical skill during an intervention can significantly hamper successful results. Read More

View Article and Full-Text PDF
April 2022
Similar Publications

Cerebral Autoregulation in Unilateral Carotid Agenesis: How Low Can We Go?

Authors:
Joana Almeida Francisco Seixas Carlos Mexedo Humberto Machado

Cureus 2022 Apr 18;14(4):e24232. Epub 2022 Apr 18.

Anesthesiology, Centro Hospitalar e Universitário do Porto, Porto, PRT.

Dysgenesis of the internal carotid artery (ICA) is a rare vascular disorder. It has a variety of different grades (agenesis, aplasia, and hypoplasia) and is more common on the right side. Although the ICA is an important vessel, most patients are asymptomatic due to collateral circulation. Read More

View Article and Full-Text PDF
April 2022
Similar Publications

Intraoperative Monitoring Cerebral Blood Flow During the Treatment of Brain Arteriovenous Malformations in Hybrid Operating Room by Laser Speckle Contrast Imaging.

Authors:
Sicai Tao Tingbao Zhang Keyao Zhou Xiaohu Liu Yu Feng Wenyuan Zhao Jincao Chen

Front Surg 2022 6;9:855397. Epub 2022 May 6.

Department of Neurosurgery, Zhongnan Hospital, Wuhan University, Wuhan, China.

Background: Hemodynamic changes caused by hybrid surgery for brain arteriovenous malformations (bAVM) are usually related to long-term lesions from "blood stealing". There are currently no viable low-cost, noninvasive procedures for assessing cerebral perfusion in the operating room. This study aims to investigate the use of intraoperative laser speckle contrast image (LSCI) software in AVM surgery. Read More

View Article and Full-Text PDF
May 2022
Similar Publications

Discovery of a trans-sellar vascular supply for the pituitary gland.

Authors:
Casey P Spinelli Joe Iwanaga Mi-Sun Hur Aaron S Dumont R Shane Tubbs

Anat Cell Biol 2022 May 23. Epub 2022 May 23.

Department of Neurosurgery, Tulane University School of Medicine, New Orleans, LA, USA.

The vasculature of the pituitary gland is discussed briefly and the details of an anatomical discovery of the vessels supplying the pituitary gland provided. Twenty latex injected cadaveric heads were dissected. Any vessels that were found to penetrate the sella turcica and travel to the pituitary gland were documented and measured. Read More

View Article and Full-Text PDF
May 2022
Similar Publications

Ruptured dural arteriovenous fistula and sinus venous thrombosis following surgical resection of a vestibular schwannoma: Case report and review of the literature.

Authors:
M Lozouet V Gilard A Nassihi J P Marie S Derrey

Neurochirurgie 2022 May 19. Epub 2022 May 19.

Rouen University Hospital, Department of Neurosurgery, 1, rue de Germont, 76000, Rouen, France.

Dural arteriovenous fistula (DAVF) is a rare vascular malformation. Strong evidence suggests that the development of DAVF in adults is acquired and multifactorial. The link between cerebral venous thrombosis and DAVF is probably explained by dynamic changes in the venous drainage pattern. Read More

View Article and Full-Text PDF
May 2022
Similar Publications
}