Hostname: page-component-78c5997874-8bhkd Total loading time: 0 Render date: 2024-11-10T05:44:09.320Z Has data issue: false hasContentIssue false

Left ventricular function in pulmonary atresia with intact ventricular septum after systemic-to-pulmonary arterial shunt

Published online by Cambridge University Press:  19 August 2008

Giuseppe Pacileo
Affiliation:
From the Department of Pediatric Cardiology, University of Naples, Monaldi Hospital, Naples
Carlo Pisacane
Affiliation:
From the Department of Pediatric Cardiology, University of Naples, Monaldi Hospital, Naples
Maria Giovanna Russo
Affiliation:
From the Department of Pediatric Cardiology, University of Naples, Monaldi Hospital, Naples
Raffaele Calabrò*
Affiliation:
From the Department of Pediatric Cardiology, University of Naples, Monaldi Hospital, Naples
*
Dr. Raffaele Calabrò, Via Bracco 71, 80133 Naples, Italy. Fax. (81) 706 2355.

Abstract

To investigate the left ventricular systolic and diastolic function in patients with pulmonary atresia with intact ventricular septum without coronary-cardiac fistulas after a modified Blalock-Taussig shunt, 14 patients (age range 15 days-16.5 months, mean 4.03±5.6 months) and eight control subjects, matched for age, body surface area and heart rate were evaluated by cross-sectional and Doppler echocardiography. The follow-up interval after palliative procedures ranged from 12 days to 16.3 months (mean 3.67±5.6 months). Compared to controls, in the group of patients the ejection fraction was decreased (61±7% vs 68±5%, p=0.022) while the left ventricular end-diastolic volume indexed for body surface area was increased (72.7±10.8 cc/m2 46.1±12 cc/m2 p=0.0001) with normal values of left ventricular mass indexed for body surface area (67.88±20.9 g/m2 vs 76±10 g/m2 p=NS). Mass-to-volume ratio was lower in patients with pulmonary atresia (0.95±0.38 vs 1.24±0.3, p=0.031). The left ventricular shape index was increased in all patients with pulmonary atresia (1.27±0.26 vs 1±0.01, p=0.009). A significant inverse correlation was noted between the ejection fraction and follow-up (r=−0.71, p=0.04). as well as between the ejection fraction and shape index (r=−0.76, p=0.048). Moreover, the patients with pulmonary atresia had decreased E/A velocity ratio (0.65±0.16 vs 1.35±0.90, p=0.009), decreased normalized peak filling rate (4.16±0.13 sv/s vs 6.88±0.68 sv/s, p=0.0001), increased peak A velocity (0.95±0.17 m/s vs 0.51±0.16 m/s, p=0.0001) and prolonged isovolumic relaxation time (46±5.4 ms vs 34±6.2 ms, p=0.0001) and deceleration time (196.4±32.2 ms vs 116±21.4 ms, p=0.0001). There was a good correlation between the normalized peak filling rate and follow-up (r=−0.80, p=0.04). These data show a progressive compromise of the left ventricular systolic and diastolic function in patients with pulmonary atresia with intact ventricular septum without ventriculocoronary fistulas who had undergone systemic-to-pulmonary arterial shunting. Thus, an earlier biventricular or Fontan type procedure should be recommended.

Type
Original Articles
Copyright
Copyright © Cambridge University Press 1994

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Calder, AL, Co, EE, Sage, MD. Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum. Am J Cardiol 1987; 59: 436442.CrossRefGoogle ScholarPubMed
2.Burrows, L, Benson, LN, Freedom, RM. Coronary angiography in pulmonary atresia with intact ventricular septum. In: Freedom, RM (ed). Pulmonary Atresia with Intact Ventricular Septum. Futura Publishing, New York, 1989, pp 207228.Google Scholar
3.Daliento, L, Scognamiglio, R, Thiene, G, Hegerty, A, Caneve, F, Anderson, RH. Morphologic and functional analysis of myocardial status in pulmonary atresia with intact ventricular septum—an angiographic, histologic and morphometric study. Cardiol Young 1992; 2: 361366.CrossRefGoogle Scholar
4.Fyfe, DA, Edwards, WD, Driscoll, DJ. Myocardial ischemia in patients with pulmonary atresia and intact ventricular septum. J Am Coll Cardiol 1986; 8: 402406.CrossRefGoogle ScholarPubMed
5.Fricker, FJ, Zuberbuhler, JR. Pulmonary atresia with intact ventricular septum. In: Anderson, RH, Macartney, FJ, Shinebourne, EA, Tynan, M (eds). Paediatric Cardiology. Churchill Livingstone, London, 1987, pp 711718.Google Scholar
6.Silverman, NH, Schiller, NB. Apex echocardiography: A two-dimensional technique for evaluating congenital heart disease. Circulation 1978; 57: 503511.CrossRefGoogle Scholar
7.Lavine, SJ, Tami, L, Jawad, I. Pattern of left ventricular diastolic filling associated with right ventricular enlargement. Am J Cardiol 1988; 62: 444448.CrossRefGoogle ScholarPubMed
8.Snider, AR, Serwer, GA. Echocardiography in Pediatric Heart Disease. Year Book Medical Publishers, New York, 1990, pp 118119.Google Scholar
9.Bowman, LK, Forrester, AL,Jaffe, CC, Mattera, J, Wackers, FJT, Zaret, BL. Peak filling normalized to mitral stroke volume: a new Doppler echocardiographic filling index validated by radionuclide angiographic techniques. J Am Coll Cardiol 1988; 12: 937943.CrossRefGoogle ScholarPubMed
10.de Leval, MR, Bull, C, Stark, J. Pulmonary atresia and intact ventricular septum: surgical management based an revised classification. Circulation 1982; 66: 272.CrossRefGoogle ScholarPubMed
11.Trusler, GA, Yamamoto, N, Williams, WG, Izukawa, T, Rowe, RD, Mustard, WT. Surgical treatment of pulmonary atresia with intact interventricular septum. Br Heart J 1976; 38: 957960.CrossRefGoogle Scholar
12.O'Connor, WN, Cottrill, CM,Johnson, GL, Noonan, JA, Tood, EP. Pulmonary atresia with intact ventricular septum and ventriculo-coronary communications: surgical significance. Circulation 1982; 65: 805809.CrossRefGoogle Scholar
13.Sauer, U, Mocellin, R, Henglein, D. Pulmonalkiappenatresic und hochgradige (kritische) pulmonalklappenstenosi mit intakien ventrikel septum. Herz 1977; 2: 398410.Google Scholar
14.Sideris, EB, Olley, PM, Spooner, E, Farina, M, Foster, E, Trusler, G, Shaher, R. Left ventricular function and compliance in pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg 1982; 84: 191199.CrossRefGoogle ScholarPubMed
15.Scognamiglio, R, Daliento, L, Razzolini, R, Boffa, GM, Pellegrino, PA, Chioin, R, Dalla Volta, S. Pulmonary atresia with intact septum: a quantitative cineangiographic study of the right and left ventricular function. Pediatric Cardiol 1986; 7: 183187.CrossRefGoogle Scholar
16.Louie, EK, Rich, S, Brundage, BH. Doppler echocardiographic assessment of impaired left ventricular filling in patients with right ventricular pressure overload due to primary pulmonary hypertension. J Am Coll Cardiol 1986; 8: 12981306.CrossRefGoogle ScholarPubMed
17.Louie, EK, Rich, S, Levitsky, S, Brundage, BH. Doppler echocardiographic demonstration of the differential effects of right ventricular pressure and volume overload on left ventricular geometry and filling. J Am Coll Cardiol 1992; 19: 8490.CrossRefGoogle ScholarPubMed
18.Bove, AA, Santamore, WP. Ventricular interdependence. Prog Cardiovasc Dis 1981; 23: 365388.CrossRefGoogle ScholarPubMed
19.Moulopulos, SD, Sarcas, A, Stametelopoulos, S. Left ventricular performance during by-pass or distension of the right ventricle. Circ Res 1965; 17: 484491.CrossRefGoogle Scholar
20.Snider, AR, Gidding, SS, Rocchini, AP, Rosenthal, A, Dick, M II, Crowley, DC, Peters, JDoppler evaluation of left ventricular diastolic filling in children with systemic hypertension. Am J Cardiol 1985; 56: 921926.CrossRefGoogle ScholarPubMed
21.Gidding, SS, Snider, AR, Rocchini, AP, Peters, J, Farnsworth, R. Left ventricular diastolic filling in children with hypertrophic cardiomyopathy: assessment with pulsed Doppler echocardiography. J Am Coll Cardiol 1986; 8: 310316.CrossRefGoogle ScholarPubMed
22.Appleton, CP, Hatle, LK, Popp, RL. Relation of transmitral flow velocity patterns to left ventricular diastolic function: new insights from a combined hemodynamic and Doppler echocardiographic study. J Am Coll Cardiol 1988; 12: 426440.CrossRefGoogle ScholarPubMed
23.Stoddard, MF, Pearson, AC, Kern, MJ, Ratcliff, J, Mrosek, DG, Labovitz, AJ. Left ventricular diastolic function: comparison of pulsed Doppler echocardiographic and hemodynamic indexes in subjects with and without coronary artery disease. J Am Coll Cardiol 1989; 13: 327336.CrossRefGoogle ScholarPubMed
24.Sholler, GF, Colan, SD, Sanders, SP. Effect of isolated right ventricular outflow obstruction on left ventricular function in infants. Am J Cardiol 1988; 62: 778784.CrossRefGoogle ScholarPubMed
25.Stone, FM, Bessinger, FB Jr, Lucas, Rv Jr, Moller, JH. Pre and postoperative rest and exercise hemodynamics in children with pulmonary stenosis. Circulation 1974; 49: 11021106.CrossRefGoogle ScholarPubMed
26.Ross, J JrCardiac function and myocardial contractility: a perspective. J Am Coll Cardiol 1983; 1: 5262.CrossRefGoogle ScholarPubMed
27.Colan, SD, Borow, KM, Neumann, A. Left ventricular endsystolic wail stress-velocity of fiber shortening relation: a load-independent index of myocardial contractility. J Am Coll Cardiol 1984; 4: 715724.CrossRefGoogle Scholar
28.Golan, SD. Noninvasive assessment of myocardial mechanics—a review of analysis of stress-shortening and stress-velocity. Cardiol Young 1992; 2: 113.Google Scholar
29.Harrison, MR, Clifton, GD, Pennell, AT, De Maria, AN. Effect of heart rate on left ventricular diastolic rransmitral flow velocity patterns assessed by Doppler echocardiography in normal subjects. Am J Cardiol 1991; 67: 622627.CrossRefGoogle ScholarPubMed
30.Choong, CY, Abascal, VM, Thomas, JD, Guerrero, JL, McGlew, S, Weyman, AE. Combined influence of ventricular loading and relaxation on the rransmitral flow velocity profile in dogs measured by Doppler echocardiography. Circulation 1988; 78: 672683.CrossRefGoogle ScholarPubMed
31.Stoddard, MF, Pearson, AG, Kern, MJ, Ratcliff, J, Mrosek, DG, Labovitz, AJ. Influence of alteration in preload on the pattern of left ventricular diastolic filling as assessed by Doppler echocardiography in humans. Circulation 1989; 79: 12261236.CrossRefGoogle ScholarPubMed