PubMed · 9117737
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I Kato, K Horike. 1996. [Vascular ring].. https://pubmed.ncbi.nlm.nih.gov/9117737/
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During the past 12 years (1985-1998), 28 infants and children were operated on here for tracheomalacia. The diagnosis was made in all using rigid bronchoscopy. During the examination the infants breathed spontaneously, but the trachea collapsed on forced expiration. Indications for surgery were repeated cyanotic spells ("dying spells") in 22, recurrent pneumonia, and inability to extubate (in 8). In 11 there were more than 1 indications. Age at surgery was from 7 days to 3 years (average 11.7 months). All 28 children underwent bronchoscopy and guided aortopexy via a left-third intercostal approach. The ascending aorta and aortic arch (and in 6 the proximal innominate artery as well) were lifted anteriorly, using 3-5 non-absorbable sutures (5.0). The sutures were placed through the adventitia of the great vessels and then passed through the sternum. Respiratory distress was significantly improved in 21. Another 2 required external tracheal stenting with autologous rib grafts, and in 1 other an internal Palmaz stent was introduced for tracheal stability. In 4 aortopexy failed, 1 of whom had tracheobronchomalacia throughout, and another 3 had laryngomalacia which required tracheostomy to relieve the respiratory symptoms. Postoperative complications were minor: pericardial effusion in 1 and relaxation of the left diaphragm in another. 1 infant subsequently died, of unknown cause 10 days after operation, after having been extubated on the 1st postoperative day. On long-term follow-up (6 months to 12 years) 25 were found free of residual respiratory symptoms and 3 remained with a tracheostomy. Thus, infants and children with severe tracheomalacia associated with severe respiratory symptoms, can be relieved by bronchoscopic guided suspension of the aortic arch to the sternum.
BACKGROUND: Multifactorial inheritance is probably involved in most cases of nonsyndromic conotruncal heart defects (CHDs), but Mendelian transmission is often suspected. RESULTS: We report on a family with recurrence of nonsyndromic CTHD in two double first cousins; i.e., two brothers married two sisters. One of the cousins (case 1) had interrupted aortic arch (IAA) type B, while the other one (case 2) had truncus arteriosus (TA) with atrioventricular canal defect (AVCD). CONCLUSIONS: Our family further supports monogenic inheritance of CTHDs. In addition, the presence of TA associated with AVCD in one of the patients confirms the higher occurrence of CTHD in families with complex TA. The absence of 22q11 microdeletion (del22q11) in our patients, as in several literature reports of familial CTHDs, supports the existence of genes different to those located on chromosome 22q11 which could be implicated in the pathogenesis of CTHDs.
We describe here a method for generating relative pressure maps from magnetic resonance velocity data in three spatial and one temporal dimension (4D). The relative pressure map calculated for pulsatile flow in a compliant phantom was shown to be consistent with independent pressure transducer measurements. The feasibility of performing 4D pressure mapping in vivo is also demonstrated.