PubMed · 5439154
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P Ostendorf, H Noltenius. 1970-03-14. [Mesaortitis luica].. https://pubmed.ncbi.nlm.nih.gov/5439154/
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Interrupted aortic arch (IAA) is often related developmentally to subaortic obstruction (SAO). When severe, SAO must be addressed in surgical management of IAA. From 1990 to 1993, 25 neonates presented for initial surgical management of IAA complexes. Associated lesions were ventricular septal defect (VSD) with or without atrial septal defect (19 patients), truncus arteriosus (3 patients), tricuspid atresia with transposition of the great arteries (1 patient), aortic atresia with VSD (1 patient), and d-transposition of the great arteries with VSD (1 patient). Overall hospital mortality was 20% (five deaths). One death was related to sepsis and two to sudden hemodynamic decompensation (a 2-kg premature infant after arch repair and VSD closure and a neonate with IAA-truncus arteriosus after arch repair and truncus repair with aortic root replacement). Two deaths were related to low cardiac output in patients with severe subaortic narrowing (< 3 mm by two-dimensional echocardiography), which was not addressed surgically. Of 10 additional patients judged preoperatively to have severe SAO, 1 underwent resection of the infundibular septum together with VSD closure and arch reconstruction, and 9 underwent a modification of Norwood's operation with arch reconstruction and proximal pulmonary artery to aortic anastomosis (7 with systemic to pulmonary artery shunts and 2 with right ventricle to pulmonary artery outflow tract reconstruction). One patient died 2 months after surgery of staphylococcal sepsis. All 9 others were discharged well. Subaortic narrowing is a physiologically important element of IAA complexes. When SAO is severe, satisfactory initial palliation can be achieved by a modification of Norwood's operation.
Pacing reduces the sub-aortic pressure gradient in hypertrophic obstructive cardiomyopathy. This phenomenon was described long ago but only recently, with the development of sophisticated pacemakers, has it become possible to use pacemakers for the long term treatment of this disease. The acute hemodynamic improvement with pacemaker treatment is well documented. The reason for this acute and chronic improvement is of multiple origin. Pacing modulates the activation sequence of the left ventricle, reduces contractility, reduces subaortic flow velocities, influences mitral valve movement and leads to remodeling of the ventricle in the long term. All these factors together explain the clinical and hemodynamic improvement in patients suffering from hypertrophic obstructive cardiomyopathy.
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