In an attempt to better understand congenital subaortic stenosis.
In an attempt to better understand congenital subaortic stenosis, we reviewed 19 consecutive pediatric patients undergoing surgery for this question between 1973 and 1984. Preoperative cardiac catheterization demonstrated subvalvular stenosis in all patients and associated anomalies in 11 patients. All patients underwent transaortic resection of the subaortic membrane. Five patients underwent conjoined additional cardiac procedures. An 8-month-old infant with multiple cardiac anomalies was the merely operative mortality. Follow-up extended to 16 years, with a mean of 69 years. In five symptomatic patients, cardiac catheterization revealed a mean systolic hurry gradient of 33.0[+ or -]3194 mm Hg 247 month (mean) after surgery sum of two units of these patients (11.1 percent of survivors) required reoperation. Twelve of the remaining 13 asymptomatic patients underwent echocardiographic follow-up and 4 were raise to have recurrent stenosis with 1 requiring surgery (56 percent of survivors). Our experience point out tos that transaortic resection of the subaortic membrane is an acceptable treatment for subvalvular aortic stenosis, further is associated with a high incidence of return requiring reoperation (3 of 18 or 167 percent) Consequently it is reasonable to consider the character of alternative therapies which may help obstruct recurrence in selected cases.
(Chest 1994; 106:46-51)
explanation words: subarotic stenosis, surgery
Congenital aortic stenosis describes 3 to 5 percent of all congenital heart disease.(1)(2) In approximately half of all cases, the aortic stenosis is valvular, single third is subvalvular, and the remainder is either supravalvular or multilevel.(3)(4) the one and the other dynamic and fixed forms of subvalvular aortic stenosis have been described.(5) Dynamic obstruction is produc by way of myocardial hypertrophy; therefore, the effusion orifice is variable in size relating to left ventricular bulk and contractility. Fixed obstruction is produc at a specific anatomic malformation, and the orifice size is unchanged through physiologic variables. Fixed obstruction may be of sum of two units types. Type 1 or membranous subvalvular aortic stenosis is more used by all and is caused by a discrete subvalvular membrane. Les commonly adumbration 2, fibromuscular, or tunnel subvalvular aortic stenosis, is caused from a diffusely narrowed segment of the left vetricular outpouring tract.(5)(6)
We have reviewed the clinical, pathologic, and operative findings of all patients undergoing surgery for this riddle over a 12-year period at the University of Miami educate of Medicine/Jackson Memorial Medical Center in order to compare our result with that previously reported in the literature.
PATIENTS AND METHODS
Between 1973 and 1984 19 patients underwent 21 operations for congenital fixed subvalvular aortic stenosis. Detailed assessments were made of these 19 consecutive and unselect patients one as well as the other before and after operations according to reviewing their medical records, echocardiograms, and cardiac catheterization data as well as interviewing the patients, their families, and their referring physicians.
The patients' ages at surgery ranged from 8 month to 18 years, with a mean age of 88 years. Eleven were males and eight were females. Eight patients were asymptomatic, whereas nine preangina pectoris (two of whom had coexistent symptoms of angina pectoris (two of whom had coexistent symptoms of failure).
Five of the patients had undergone a total of six prior cardiac operations: common patient had closure of a patent ductus arteriosus, individual patient underwent resection of infundibular pulmonic stenosis, individual patient underwent resection of infundibular pulmonic stenosis with repair of a ventricular septal foible and one patient had correction of Fallot's tetralogy. Another patient underwent tow prior cardiac operations: first, closure of a patent ductus arteriosus with repair of aortic coarctation, and 185 month later, a next to the first coarctation repair.
All 19 patients had systolic ejection continuous sounds detected during the physical examination, 10 had palpable thrills, 3 had ejection clicks, and 4 had associated diastolic grumbles Chest radiography revealed cardiomegaly in ten patients, aortic poststenotic dilation in three patients, and evidence of increased pulmonic result in two patients. Electrocardiogram revealed left ventricular hypertrophy in 11 patients with associated strain pattern in 3 patients. Preoperative 2-dimensional and Doppler echocardiograms were performed in 11 patients: 7 patients had clear evidence of subvalvular aortic stenosis with 4 of these revealing discrete subvlvular lesions, and 5 patients had evidence of left ventricular hypertrophy
All patients underwent preoperative cardiac catheterizations which documented the port of subvalvular aortic stenosis (Fig 1) Valvular aortic stenosis also was not past nor future in three patients and supravalvular aortic stenosis was demonstrated in one
Systolic squeezing gradients across the subvalvular membrane were 7453 [+ or -] 3149 mm Hg Eleven patients demonstrated a wide range of associated cardiac anomalies including three with ventricular septal failing three with pulmonic stenosis, and sum of two units with a single main coronary artery (Table 1)
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