Survey
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
Electrocardiography wikipedia , lookup
Management of acute coronary syndrome wikipedia , lookup
Coronary artery disease wikipedia , lookup
Heart failure wikipedia , lookup
Antihypertensive drug wikipedia , lookup
Cardiothoracic surgery wikipedia , lookup
Mitral insufficiency wikipedia , lookup
Cardiac surgery wikipedia , lookup
Quantium Medical Cardiac Output wikipedia , lookup
Lutembacher's syndrome wikipedia , lookup
Atrial septal defect wikipedia , lookup
Dextro-Transposition of the great arteries wikipedia , lookup
Commentary ANOMALOUS PULMONARY VENOUS RETURN WITH STENOSIS IN COMPLEX LEFT HEART DEFECT – LETHAL ANOMALY – DESCRIPTION OF 3 CASES BY E. GÓRA & CO-AUTHORS. Author: Prof. Andrzej Rudziński Head of the Department of Pediatric Cardiology Jagiellonian University, Cracow Prenat Cardio. 2013 Mar;3(1)28-29 DOI 10.1515/pcard-2015-0009 Echocardiographic prenatal diagnosis is an important and increasingly popular method available for the diagnosis of birth defects and heart disease in children. It is particularly important in fetuses with complex defects requiring urgent surgery after birth and / or intervention. Prior information about such cases allows mothers to be referred for delivery to maternity centers which will secure pre-treatment and efficient transfer of the newborn to the closest possible pediatric cardiac and cardiothoracic surgery center. This reduces the time to undertake further investigation and necessary treatment which ultimately reduces the risk of various complications. The childs chances of survival increase and costs of treatment can be reduced. In very rare cases of complex defects, which do not currently have any treatment options, early detection allows the parents to get used to the thought that the defect is terminal and allow room for palliative and conservative therapy. Therefore, I consider the authors work to be important and very interesting for cognitive reasons and those stated above. The paper presents three cases of fetuses with left heart defects and abnormal confluence of the pulmonary veins and the carotid, determining the lethal nature of this constellation. Abnormal pulmonary venous flow coexists with hypoplastic left heart syndrome in about 5% of cases. It may be accompanied by restriction or even complete absence of the foramen ovale. Often in these cases there is a patent left cardinal vein draining into the innominate vein . Intracardiac type with venous return entering the right atrium is quite rare. Pulmonary veins and the left atrium develop separately about 25 - 27 days of gestation. The primary pulmonary vein system is part of the celiac plexus and initially connects to the cardinal vein system and umbilical and vitilline veins. In short, the right-hand cardinal system is transformed into the right sinus venosus, from which the right vena cava superior is formed (R-SVC) and the vena azygos, and the left-hand side evolves into the left sinus venosus, from which the left vena cava superior and coronary sinus are formed, and from the umbilical and vitilline system the inferior vena cava, ductus venosus and portal vein are formed. The common pulmonary vein draining blood from the primary pulmonary veins forms probably from the distention of the upper posterior wall of the left atrium, or the pulmonary veins confluense (27 - 29 of gestation). However, the primary connection of the pulmonary veins, the cardinal vein and the umbilical and vitilline systems overgrow. The development of the pulmonary vein and its incorporation into the left atrium determines the proper confluence, and any disturbances in the process lead to anomalies in the form of partial or total abnormal pulmonary venous return into systemic veins (by residual elements of development), or directly to the right atrium2, 3 . Defects in the process of incorporation of the primary common pulmonary vein to the left atrium is also responsible for the creation of the- cor triatriatum sinistrum. Her late atresia (very rare when the collateral venous circulation channels are already obstructed) makes the individual pulmonary veins open up to blind diverticuli with no connection to the left atrium or systemic veins4. This developmental change is beyond any intervention. Narrowing of the outlet of the pulmonary veins can occur with the proper connection to the left atrium, as well as in outlet anomalies, mainly in the infradiaphragmatic type. In addition to the type of return and the associated defects the fate of the patient is determined by the location and nature of the stenosis (pressure from outside, internal changes, limited or diffuse narrowing) 3 Stenosis by external factors is possible to eliminate radically. However, narrowing by other reasons faces considerable difficulties and poor prognosis. This is due to the tendency to relapse and to progression of Corresponding author: [email protected] Unauthenticated Download Date | 6/17/17 6:28 AM 28 PRENAT CARDIO. 2013 MAR;3(1):28-29 vascular changes in the vessels lumen, despite emergency surgery, and expansion and / or stenting of the stenosis leading to the development of postcapillary pulmonary hypertension and right ventricular failure. External abnormal narrowing in the total anomalous return results from the local oppression of the collective channel. This applies for example to vertical veins draining into the vein innaminate, in the course of the left bronchus and the left branch of the pulmonary artery, pulmonary vein, or the outlet of the R-SVC (due to stenosis at the branch running between the right lung and trachea). In the infradiaphragmatic type narrowing is a result of processes related to its natural construction. Runoff into the portal vein and its tributaries encounters resistance due to the presence of hepatic sinusoid. The impediment may be caused by the channels length alone, as well as a restrictive foramen ovale (which was also found in the material of the authors). Significant pulmonary hypertension and venous stasis dominates In total abnomolous pulmonary venous return with stenosis without accompanying defects . This quickly leads to the development of right heart failure and decrease in left ventricular ejection through reducing blood flow to the left heart. Patent ductus arteriosus after birth is the reason for the right-left shunt complementing systemic ejection. Clinical picture (severe shortness of breath, galloping tachycardia, hepatomegaly, cyanosis within 24-36 hoursof birth) is similar to the image presented in supradiaphragmatic aortic coarctation or persistent fetal circulation in the newborn, which requires urgent differential diagnosis. In hypoplastic left heart syndrome with total abnormal pulmonary venous return without restriction, after birth (due to a decrease in pulmonary resistance) there is a growing increase in pulmonary flow and reduced systemic flow (as most of the right side outflow enters the pulmonary circulation, and little through the ductus arteriosus into the descending aorta). Andrzej Rudziński pulmonary venous return with obstruction, shortly after birth the child's condition rapidly deteriorates. Obstruction of the pulmonary veins return causes severe hypertension and pulmonary stasis and reduces the inflow of oxygenated blood to the right ventricle. This results in a decrease in systemic circulation, into which prevails blood (mainly through the ductus arteriosus) with slightly higher saturation than that of the venous system. Severe dyspnea and cyanosis dominates (oxygen saturation of arterial blood is low, <70%). Symptoms of cardiac failure occur rapidly or are already present. Therefore detailed assessment of the type of anomaly, location and nature of the obstruction is extremely necessary. In the described cases treatment of anomaly was impossible, which disqualified from any surgical treatment / intervention. Thus, earlier prenatal echocardiographic diagnosis in these cases was very helpful in the initial evaluation of such changes and their prognostic significance References: 1. Selim MA, Chin AJ, Norwood WI: Patterns of anomalous pulmonary venous connection/drainage in hypoplastic left heart syndrome: diagnostics role of doppler color flow mapping and surgical implications. J Am Coll Cardiol. 1992; 19:135-141 2. Ward KE, Mullins CE: Anomalous pulmonary venous connections, vein stenosis, and atresia of the common vein. In: Garson A, Bricker JT, Fisher DJ, Neish SR, eds: The Science and Practice of Pediatric Cardiology. Baltimore, Md: Williams and Wilkins; 1998:1431–1461 3. Breinholt JP, Hawkins JA, Minich L, at al: Pulmonary vein stenosis with normal connection: associated cardiac abnormalities and variable outcome. Ann Thorac Surg. 1999; 68:164–168 4. Brown DW, Geva T: Anomalies of pulmonary veins. In: Allen HD, Driscoll DJ, Shaddy RE, Feltes TF, eds. Heart disease in infants children and adolescents including the fetus and young adults. 8th ed. Wolters Kluwer/Lippincott Williams&Wilkins 2013. 809-839 In children, however, the oxygen saturation of arterial blood normally exceeds 90%. It is necessary to maintain the patency of the ductus arteriosus (Prostin) and the implementation of pharmacotherapy decreasing peripheral resistance (nitroprusside, Corotrop). Sometimes it is necessary to intubate and use respiratory therapy in terms of increasing pulmonay resistance. Early surgical treatment is necessary (surgery by Norwood method/ Sano or hybrid treatment (expanding / stenting the foramen ovale, the ductus arteriosus stent, "banding" of the pulmonary artery). Correction of pulmonary venous return (not including supradiaphragmatic type) is not necessary in such cases . In hypoplastic left heart syndrome with total abnormal 29 Unauthenticated Download Date | 6/17/17 6:28 AM