Hypoplastic’s Left-Sherz-Synddrom-Wikipedia

At the Hypoplastic left-heart syndrome ( HLHS ), also Leftherz hypoplasia syndrome called ( Hypoplastic = too small, underdeveloped ), it is several malformations of the heart and the connected main artery (Aorta). The hypoplastic left heart is characterized by a complete duct addiction (i.e. the blood flow through a small vascular connection, the duct arteriosus botalli). The reason for this are:

Morphology of the hypoplastic left-heart syndrome

• A high -grade aortic valve stenosis or aortic valve athresia (narrowing or absence/closure of a heart valve between the aorta and the left heart chamber = ventricle)
• and/or a mitral valve stenosis or mitral valve atresia (narrowing or absence/closure of the flap between the left atrium and the left main chamber)
• With high grade hypoplasia (= underdevelopment) of the Aorta Ascendens (the ascending part of the Large body artery , from which the coronary arteries arise) (2–4 mm, normal 10–12 mm) in type I
• and in type II (different appearance of the heart defect in different forms) with a normal diameter of the Aorta Ascenden
• and very small (hypoplastic) or missing left ventricle (which cannot develop due to the mitral valve misalignment))
• and often at the same time in the same hypoplastic left atrium = atrium

Endocardial fibrosis (thickening of the rainwand without functional benefits) of the left ventricle and atrium can also occur regularly. Most of the time, the ventricular septum (heart septum at the chamber level) is intact, while an open foramen oval (= opening between the right and left heart rate, which is called foramen oval (= ovales hole “) due to its appearance) or a real atrial septal defect (ASD) the abstrom of the pulmonary venous (flowing from the lung vein) blood from the left atrium into the right atrium. In up to 20% of cases there is a preductal aortic stenosis (= narrow of the aorta before the confluence of the duct arteriosus). The right heart structures are mostly enlarged (enlargement/hypertrophy of the right atrium and ventricular).

The cause of innate syndromes or mutations are also possible.
Mutations in the following genes were described: ^[first] Gja1 (6Q22.31), Hand1 (5Q33.2) ^[2] and NKX2-5 (5Q35.1) ^[3]

There is a connection with some syndromes: ^[4]

In contrast to the regular blood flow of a literally developed heart, the blood of the lung veins first flows over the left atrium when it comes to HLHS. The existing advance (foramen ovals), which ensures the child’s survival, allows the blood to mix with non -oxygen -saturated blood over the right atrium. This is called medically left-right shunt. Furthermore, the mixed blood flows from the right ventricle into the pulmonary artery and further over the Ductus Arteriosus Botalli back into the aorta descenden (descending part of the aorta, further divided into the breast aorta and the abdominal aorta). At the same time, the (underdeveloped) aorta Ascendens (part of the aorta from which the coronary arteries arise) and thus into the coronary arteries flows over the open duct. This process is called “Retrograde Perfusion”.

Children with this clinical picture are also referred to as “Blue Baby”.

If a hypoplastic left-heart syndrome is ensured, there are various approaches to treatment today:

1. The child is left to the so -called “natural course” without medical and operational care. This means that it usually dies within a few days or hours, only in exceptional cases in a few weeks.
2. The 3-stage surgical palliation.
   • Classic Norwood operation, also called Norwood procedure
     • This surgical technique initially encloses a first life-sustaining operation, the Norwood Stage1 operation, whereby the underdeveloped aorta is first expanded by means of a plastic patch. For this purpose, the right ventricle is also connected to the aorta and a shunt (= plastic tube) for the lung circuit was created. This operation takes place using the heart-lung machine and in the condition of the cardiac arrest.
     • The Norwood Stage2 operation, also called Glenn anastomosis (or “hemi-fontan”), causes a connection between the upper hollow venue to the pulmonary artery. The child receives a safer life situation through this operation, since the shunt of the first operation can now be removed. Treatment with anticoagulant medication – such as cumarines – is no longer necessary in most cases.
     • Norwood-Stage3-Operation = complete Fontan (TCPC): With this operation step, the lower hollow vein is now also connected to the pulmonary artery, so that a complete circulatory separation is achieved.
   • Alternatively Gipes-Procedure (Hybrid-on)
     • This is a so -called hybrid intervention by children’s heart surgeons and children’s cardiologists. First of all, the catheter intervention of a stent is carried out into the ductus arteriosus to ensure the perfusion of the body cycle. In the following, a bilateral bound of the pulmonary arteries for throttling the pulmonary blood flow is surgically created. The operation can initially take place without a heart-lung machine. ^[5]
     • In the course of Norwood-Stage2 operation with reconstruction of the underdeveloped aorta (see above).
3. As a rare last option: a heart transplantation

Although the HLHS is a surely serious cardiac misalignment complex, the experience of recent years shows that the treatment of this heart defect in the specialized heart centers now offers good treatment prospects. The experiences from America show that there are already adults who were once born with HLHs and who received a good to very good quality of life through Norwood procedure. In the meantime, the HLHS in America is no longer necessarily an indication for abortion. On the contrary, there is even a treatment obligation for children with this malformation, since with adequate treatment, these children can be helped to live a normal life with a good quality of life.

The different ways of viewing between Germany and America also stir because, for. T. still incorporate the numbers from the beginning of this operation technology in Germany into the mortality rates, which thus show a falsified image of today’s reality. Through the use of routine children’s heart surgeons, improved operating techniques and refined examination methods, a large part of the children operated today has much better long-term forecasts than at the beginning of this surgical engineering in the 1980s. The last option also remains the possibility of a heart transplant, which can now be carried out with good success. For HLHs, there are different treatment approaches that open up the possibility of a fulfilling life with a good quality of life for the child.

Back to heart defect descriptions

1. ↑ D. R. Fulton: “Hypoplastic left heart syndrome”, 2021, Up To Date
2. ↑ Hypoplastic left heart syndrome 1.  In: Online Mendelian Inheritance in Man . (English)
3. ↑ Hypoplastic left heart syndrome 2.  In: Online Mendelian Inheritance in Man . (English)
4. ↑ J. A. Feinstein, D. W. Benson, A. M. Dubin et al.: Hypoplastic left heart syndrome: current considerations and expectations. In: Journal of the American College of Cardiology. Band 59, Number 1 Suppl, January 2012, S. S1–42, doi: 10.1016/j.jacc.2011.09.022 , PMID 22192720 , PMC 6110391 (Free full text) (review).
5. ↑ Hakan Akintürk, Ina Michel-Behnke, Klaus Valeske, Matthias Mueller, Josef Thul: Hybrid Transcatheter–Surgical Palliation . In: Pediatric Cardiology . Band 28 , No. 2 , 1. April 2007, ISSN  1432-1971 , S. 79–87 , doi: 10.1007/s00246-006-1444-7 .

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