Early and intermediate-term (10-year) results of surgery for univentricular atrioventricular connection (“single ventricle”)

Stefanelli, Guglielmo; Kirklin, John W.; Naftel, David C.; Blackstone, Eugene H.; Pacifico, Albert D.; Kirklin, James K.; Soto, Benigno; Bargeron, Lionel M.

doi:10.1016/s0002-9149(84)80213-1

Cited by 76 publications

(20 citation statements)

References 25 publications

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“…In patients designated to be at "low risk," the mortality has been reported to be as low as 3.7-17%, with relatively low morbidity. [2][3][4][5][6][7][8] In patients who are considered to be at greater risk, however, this procedure carries a mortality risk ranging between 17% and 75%, with significantly increased morbidity. [9][10][11][12][13] In an attempt to decrease the morbidity and mortality of this operation in the high-risk patient, Laks et al14,15 described a modification of the technique that entailed the creation of an externally adjustable interatrial communication to provide a controlled right-to-left shunt in the period immediately after surgery.…”

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confidence: 99%

Hemodynamic evaluation before and after closure of fenestrated Fontan. An acute study of changes in oxygen delivery.

et al. 1992

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BACKGROUND Acute changes in hemodynamics and oxygen delivery accompanying temporary occlusion of atrial defects in 14 patients after a fenestrated Fontan procedure were evaluated at a median interval of 32 days after surgery to identify candidates for permanent transcatheter closure of their defects. METHODS AND RESULTS Patients ranged in age from 9 months to 33 years and in weight from 7.9 to 69 kg. Right atrial (RA), left atrial (LA), and aortic pressures, mixed venous (SmvO2) and aortic (SaO2) oxygen saturation, and whole-body oxygen consumption (VO2) were measured, and systemic blood flow (Qs), systemic oxygen transport (SOT), and oxygen extraction were calculated before and after occlusion. SmvO2, VO2, and RA pressures did not change, but SaO2 increased from 84 +/- 6% to 95 +/- 3% (p less than 0.05), and LA pressures fell from 5.1 +/- 3.6 to 3.7 +/- 2.2 mm Hg (p less than 0.05). Qs fell from 2.4 +/- 0.7 to 1.8 +/- 0.41.min-1.m-2 (p less than 0.05), SOT fell from 425 +/- 154 to 366 +/- 112 ml.O2.min-1.m-2 (p less than 0.05), and oxygen extraction increased from 0.40 +/- 0.12 to 0.46 +/- 0.13 (p less than 0.05). Only one patient did not undergo definitive closure of his defect because of a marked decrease in Qs and SOT with a significant rise in RA pressure. CONCLUSIONS Although delayed closure of an atrial defect in these patients improved morbidity and mortality, the complete separation of the venous and systemic circulations was accomplished at the expense of decreased Qs and oxygen delivery despite the improved level of arterial oxygenation. The resting levels of oxygen extraction are elevated and will be associated with decreased exercise capability. The long-term benefits of closure of these fenestrations are yet to be shown.

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confidence: 99%

Hemodynamic evaluation before and after closure of fenestrated Fontan. An acute study of changes in oxygen delivery.

et al. 1992

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“…Furthermore, the results of definitive surgery for this type of single ventricle are still discouraging. 12 13 The cause of these less than satisfactory results may partly be the higher incidence of associated anomalies in patients with single right ventricle. 14 Ventricular dysfunction may be a more important cause of this difficulty in clinical management, but this has not yet been proven.…”

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Quantitative cineangiographic analysis of ventricular volume and mass in patients with single ventricle: relation to ventricular morphologies.

et al. 1988

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With the use of biplane selective ventriculography, the ventricular volume, ejection fraction, and ventricular mass were evaluated in 28 patients with a single ventricle, and those with the left ventricular type (LV type, 12 patients) and right ventricular type (RV type, 16 patients) were compared. There were no significant differences in terms of age, hemoglobin, systemic oxygen saturation, or pulmonary-to-systemic flow ratio in the two groups. No patients with atrioventricular valve regurgitation were included. The ventricular cavity volume was calculated by the area-length method. The ventricular mass volume was determined as the shell volume created by subtracting the ventricular cavity volume from the total ventricular volume calculated by adding the free wall thickness to the chamber dimensions. The ventricular mass volume was converted to mass by multiplying by the gravity of the heart muscle. There was no significant difference between patients with the LV type and RV type of single ventricle with respect to the end-diastolic ventricular vclume (188 + 53 and 179 + 61 ml/m2 in LV and RV types, respectively), end-systolic volume (88 + 31 and 84 + 27 ml/m2), or ejection fraction (0.54 + 0.06 and 0.52 + 0.06). The following four indexes of the ventricular mass were significantly (p < .001) lower in patients with the RV type of single ventricle: ventricular wall thickness (3.9 ± 1.2 mm in RV type vs 6.9 ± 1.9 mm in LV type), ratio of the ventricular wall thickness to the transverse diameter of the ventricle (6.8 + 1.9% vs 12.1 ± 2.4%), ventricular mass (87 ± 35 vs 160 ± 47 g/m2), and ratio of ventricular mass to end-diastolic volume (0.48 ± 0.11 vs 0.88 + 0.17 g/ml). There was a positive linear correlation between the ventricular mass index and the pulmonary-to-systemic blood flow ratio in patients with LV type (r = .71, p < .01) but no correlation was seen in those with RV type. These results suggest that there is inadequate ventricular hypertrophy (insufficient ventricular mass to ventricular volume) in patients with the RV type of single ventricle compared with that in those with the LV type and this may lead to abnormal contractile state and poor adaptation of ventricular function in patients with the RV type of single ventricle.

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“…[2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17] We have previously suggested that the recognition of subaortic stenosis in the patient with a univen-THERAPY AND PREVENTION-CONGENITAL HEART DISEASE tricular heart usually implies a discordant ventriculoarterial connection (or transposition of the great arteries) and that the documentation of subaortic stenosis usually follows pulmonary artery banding.8 9 12 13 Furthermore, we have stated that pulmonary artery banding may be causal to the genesis of subaortic stenosis or at the very least may accelerate its development.…”

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Subaortic stenosis, the univentricular heart, and banding of the pulmonary artery: an analysis of the courses of 43 patients with univentricular heart palliated by pulmonary artery banding.

Freedom¹,

Benson²,

Smallhorn³

et al. 1986

Circulation

150

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Subaortic stenosis is well known to complicate the clinical course of patients with single ventricle or univentricular hearts, and we have previously suggested that the development of subaortic stenosis in such patients may be causal to and/or accelerated by previous banding of the main pulmonary trunk. To further define the relationship between banding of the pulmonary artery in patients with univentricular hearts and the development of subaortic stenosis, we examined the morphologic substrate and timing of the development of subaortic stenosis in 43 patients seen at our institution from January 1, 1970, through June 30, 1985. These 43 patients include all patients in this period with an unequivocal univentricular heart whose longitudinal data was available for follow-up. We excluded patients who died within 1 week of surgery, patients lost to follow-up, and patients with evidence of subaortic stenosis before banding. Thirty-one of 43 patients (72. 1 %) developed subaortic stenosis subsequent to banding of the main pulmonary artery. The mean age at banding of those patients who developed subaortic stenosis was 0.21 years, and subaortic stenosis was recognized at a mean age of 2.52 years. For the specific cohort of patients whose ventricular morphology was a main chamber of left ventricular type supporting the pulmonary artery and a rudimentary right ventricle supporting the transposed aorta (32 patients), 27 developed subaortic stenosis (84.4%). Subaortic stenosis in the classic form of single ventricle usually results from progressive restriction of a wholly muscular interventricular communication. Banding of the pulmonary artery by producing myocardial hypertrophy undoubtedly accelerates the potential for subaortic stenosis in these patients. Furthermore, one must realize that subaortic stenosis may be present in the absence of a resting pressure gradient, and such subaortic stenosis can usually be unmasked by stimulation with isoprenaline. Finally, one must be guarded in advocating banding of the pulmonary artery in patients with single ventricle, realizing that subaortic stenosis strongly influences the outcome of more definitive surgery in these patients. Circulation 73, No. 4, 758-764, 1986. THE NATURAL HISTORY of patients with single ventricle (one-ventricle hearts, univentricular hearts, univentricular atrioventricular connection) has been irrevocably altered by those diverse surgical procedures that (1) augment pulmonary blood flow, (2) reduce pulmonary blood flow, (3) augment atrial mixing when intracardiac streaming is disadvantageous, or (4)

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Early and intermediate-term (10-year) results of surgery for univentricular atrioventricular connection (“single ventricle”)

Cited by 76 publications

References 25 publications

Hemodynamic evaluation before and after closure of fenestrated Fontan. An acute study of changes in oxygen delivery.

Hemodynamic evaluation before and after closure of fenestrated Fontan. An acute study of changes in oxygen delivery.

Quantitative cineangiographic analysis of ventricular volume and mass in patients with single ventricle: relation to ventricular morphologies.

Subaortic stenosis, the univentricular heart, and banding of the pulmonary artery: an analysis of the courses of 43 patients with univentricular heart palliated by pulmonary artery banding.

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