Hypoplastic left heart syndrome

Called hypoplastic left heart syndrome ( HLHS ), also Linksherzhypoplasie syndrome ( hypoplastic = too small, underdeveloped ), is more associated with each other occurring malformations of the heart and of the connected main artery ( aorta). The hypoplastic left heart is a full Ductusabhängigkeit (ie the blood flow through a small tube connection, the ductus arteriosus ) characterized. Reasons for this are:

• A high-grade aortic valve stenosis or Aortenklappenatresie (narrowing or absence / closing of a heart valve between the aorta and left ventricle = ventricle)
• And / or mitral stenosis, or Mitralklappenatresie (narrowing or absence / closing the valve between the left atrium and left main chamber )
• With severe hypoplasia ( underdevelopment = ) of the ascending aorta ( the ascending part of the great body artery, from which the coronary arteries originate ) (2-4 mm, normal 10-12 mm ) for type I
• And type II ( different appearance of the heart defect to varying degrees ) with normal diameter of the ascending aorta
• And very small ( hypoplastic ) or absent left ventricle (which is due to the Mitralklappenfehlbildung can not even develop )
• And often coexisting hypoplastic left atrium = Atrium

Also an endocardial fibrosis (thickening of the heart wall without functional benefits) of the left ventricle and atrium may occur regularly way. Usually the ventricular septum ( septum to chamber level ) is intact, while a patent foramen ( called = opening between the right and left atrium, which on the basis of their appearance foramen ovale ( = " oval hole " ) ) oval or a true atrial septal defect (ASD ) the effluent from the pulmonary venous ( flowing from the pulmonary vein ) allows blood from the left atrium into the right atrium. In up to 20% of cases there is a preductal coarctation ( = narrowness of the aorta before the opening of the ductus arteriosus ). The right heart structures are mostly compensatory enlarged ( enlargement / hypertrophy of the right atrium and ventricle ).

Physical processes / pathophysiology

In contrast to the regular blood flow of the heart flows in the proper developed HLHS, first the blood of the pulmonary veins from the left atrium. The existing gap atrium ( the foramen ovale), which ensures the survival of the child can, that mixes the blood via the right atrium with no oxygen-saturated blood. This is called medically left-right shunt. Next then flows from the right ventricle, the mixed blood into the pulmonary artery and further via the ductus arteriosus into the aorta again decendens ( descending part of the aorta, further divided into the thoracic aorta and the abdominal aorta). At the same time flows through the patent ductus arteriosus opposite to the normal direction of flow in the blood ( under development ), ascending aorta ( part of the aorta from which the coronary arteries arise ) and thus into the coronary arteries. This process is called " retrograde perfusion ".

Children with this disease are also known as " blue baby ".

Treatment

When seized diagnosis of hypoplastic left heart syndrome, there are now various approaches to treatment:

• This surgical technique initially encloses a first life-saving surgery, the Norwood - Stage1 - operation, where first the underdeveloped aorta is extended by a Kunststoffflickens. For this purpose, the right ventricle to the aorta is associated with this operation at the same time and created a shunt ( = plastic tube ) for the lung perfusion. This operation is performed using a heart - lung machine and in a state of cardiac arrest.
• The Norwood - Stage2 - operation, also called Glenn anastomosis (or " hemi- Fontan ") causes a connection to the superior vena cava to the pulmonary artery. The child receives by this operation a safer living situation, since the first surgery, the shunt can be removed now. Treatment with anticoagulant drugs - such as coumarins - is no longer necessary in most cases.
• Norwood - Stage3 - operation = complete Fontan ( TCPC ): in this operation step a connection is now also the inferior vena cava to the pulmonary artery, providing a complete loop separation is achieved.

Forecast

Although the HLHS is certainly a serious heart malformation complex, the experience of recent years shows that in the specialized heart centers in the treatment of this heart defect now offers good prospects of treatment. The experience from America show that there are already adults who once were born with HLHS and received by Norwood Procedure a good to very good quality of life. Meanwhile, the HLHS in America is no longer necessarily an indication for abortion. On the contrary, there is even a compulsory treatment for children with this malformation, as is to help these children with adequate treatment to a normal life with good quality of life.

The differences in the approach between Germany and America also, therefore, that in some cases the figures from the beginnings of this surgical technique in Germany still incorporated with in the mortality rates, which thus exhibit a distorted picture of today's reality stir. Through the use of routine pediatric cardiac surgeons, improved surgical techniques and investigative methods, a large part of today's children operated much better long-term forecasts than at the beginning of this surgical technique in the 1980s. The last option also remains the possibility of a heart transplant, which can already be performed with good results now. For the HLHS so there are various treatment approaches that open up the child the opportunity to live a fulfilled life with good quality of life.

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