The ventricle of a functionally univentricular heart is known to be dilated, hypertrophic and hypocontractile before and after completion of a Fontan type of circuit. Ventricular dysfunction can be due to the congenital malformation itself, previous surgical conditions, or the very abnormal working conditions of the ventricle at the various stages of palliation. Because of the very different types of ventricle, it remains difficult to assess size, shape, hypertrophy, stress, strain, contraction and relaxation. The ventricle after birth tries to adapt to different and very abnormal circulations, evolving from overloaded and overstretched while shunted, to underloaded and overgrown after a Fontan type of circulation.
Key words:
Cavopulmonary anastomosis - Pathophysiology
References
-
1
Graham T, Erath H, Boucek R, Boerth R.
Left ventricular function in cyanotic congenital heart disease.
Am J Cardiol.
1980;
45
1231-1236
-
2 Rudolph A M. Congenital Heart Disease. Chicago; Year Book Medical Publishers 1974: 124-126
-
3
Dick M, Fyler D C, Nadas A S.
Tricuspid Atresia: clinical course in 101 patients.
Am J Cardiol.
1975;
36
327-337
-
4 Graham T Jr, Friesinger G. Complex cyanotic congenital heart disease. In Roberts W (ed.) Adult Congenital Heart Disease. Philadelphia; FA Davis Company 1987: 541-566
-
5
Fontan F, Baudet E.
Surgical repair of tricuspid atresia.
Thorax.
1971;
26
240-248
-
6 Chaoussat A, Fontan F, Besse F, Vallot F, Chauve A, Bricaud H. Selection criteria for Fontan's procedure. In: Anderson R, Shinebourne E (eds.) Paediatric Cardiology. Edinburgh; Churchill Livingstone 1978: 559-566
-
7
Matsuda H, Kawashima Y, Hirose H, Nakano S, Sakakibara Y, Kishimoto H, Miura T, Sano T.
Modified Fontan operation for single ventricle with common atrium and abnormal systemic venous drainage: usefulness of an additional superior vena cava to pulmonary artery anastomosis.
Pediatr Cardiol.
1987;
8
43-46
-
8
Gewillig M, Lundström U, Wyse D, Bull K, Deanfield J.
Is a subpulmonary ventricle in a Fontan circulation a Trojan horse?.
Brit Heart J.
1990;
64
104
-
9
Yacoub M, Radley-Smith R.
Use of a valved conduit from right atrium to pulmonary artery for “correction” of single ventricle.
Circulation.
1976;
54 (III)
63-70
-
10
Gale A, Danielson G, McGoon D, Wallace R, Mair D.
Fontan procedure for tricuspid atresia.
Circulation.
1980;
62
91-96
-
11
Laks H, Williams W, Hellenbrand W, Freedom R, Talner N, Rowe R, Trusler G.
Results of right atrial to right ventricular and right atrial to pulmonary artery conduits for complex congenital heart disease.
Ann Surg.
1980;
192
382-389
-
12
Coles J, Leung M, Kielmanowicz S, Freedom R, Benson L, Rabinovitch M, Sherret H, Dasmahapatra H, Trusler G, McLaughlin P, Williams W.
Repair of tricuspid atresia: utility of right ventricular incorporation.
Ann Thorac Surg.
1988;
45
384-389
-
13
Lee C, Schaff H, Danielson G, Puga F, Driscoll D.
Comparison of atriopulmonary versus atrioventricular connections for modified Fontan/Kreutzer repair of tricuspid valve atresia.
J Thorac and Cardiovasc Surg.
1986;
91
1038-1048
-
14
Gussenhoven W, The H, Schippers L, Bos E, Roelandt J, Ligtvoet C.
Growth and function of the right veniricular outflow tract after Fontan's procedure for tricuspid atresia: a two-dimensional echocardiographic study.
Thorac Cardiovasc Surgeon.
1986;
34
236-239
-
15
Bull C, Macartney F J, Horvath P, Almeida R, Merill W, Douglas J, Taylor J F N, De Leval M R, Stark J.
Evaluation of long-term results of homograft valves in extracardiac conduits.
J Thorac and Cardiovasc Surg.
1987;
94
12-19
-
16
Kreutzer G.
Atriopulmonary anastomosis.
J Thorac Cardiovasc Surg.
1982;
83
427-436
-
17
Molina J, Wang Y, Lucas R, Moller J.
The technique of the Fontan procedure with posterior right atrium-pulmonary artery connection.
Ann Thorac Surg.
1985;
39
371-379
-
18
Puga F, Chiavarelli M, Hagler D.
Modifications of the Fontan operation applicable to patients with left atrioventricular valve atresia or single atrioventricular valve.
Circulation.
1987;
76 (suppl III)
53-60
-
19
Matsuda H, Kawashima Y, Kishimoto H, Hirose H, Nakano S, Kato H, Taniguchi K, Nishigaki K, Tesuya S, Ogawa M.
Problems in the modified Fontan operation for univentriculat heart of the right ventricular type.
Circulation.
1987;
76 (III)
45-52
-
20
Humes R, Feldt R, Porter C, Julsrud P, Puga F, Danielson G.
The modified Fontan operation for asplenia and polysplenia syndromes.
J Thorac Cardiovasc Surg.
1988;
96
212-218
-
21
Matsuda H, Kawashima Y, Takano H, Miyamoto K, Mori T.
Experimental evaluation of atrial function in right atrium-pulmonary artery conduit operation for tricuspid atresia.
J Thorac and Cardiovasc Surg.
1981;
81
762-767
-
22
Jonas R, Castaneda A.
Modified Fontan procedure: atrial baffle and systemic venous to pulmonary artery anastomotic techniques.
J Cardiac Surg.
1988;
3
91-96
-
23
Matsuda H, Kawashima Y, Hirose H, Nakano S, Kishimoto H, Sano T.
Evaluation of total cavopulmonary shunt operation for single ventricle with common atrioventricular valve and left isomerism.
Am J Cardiol.
1986;
58
180-182
-
24
De Leval M, Kilner P, Gewillig M, Bull C.
Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience.
J Thorac Cardiovasc Surg.
1988;
96
682-695
-
25
Van Haesdonck J, Mertens L, Montas G, Sizaire R, Purnode B, Daenen W, Crochet M, Gewillig M.
Comparison by computerised numeric modelling of energy losses in different Fontan connections.
Circulation.
1995;
92 II
322-326
-
26
Glenn W.
Superior vena cava-pulmonary artery anastomosis.
Ann Thorac Surg.
1984;
37
9-15
-
27
Boruchow I, Swenson E, Elliott L, Bartley T, Wheat M, Schiebler G.
Study of the mechanisms of shunt failure after superior vena cava-right pulmonary artery anastomosis.
J Thorac Cardiovasc Surg.
1970;
60
531-539
-
28
Cloutier A, Ash J, Smallhorn J, Williams W, Trusler G, Rowe R, Rabinovitch M.
Abnormal distribution of pulmonary blood flow after the Glenn shunt or Fontan procedure: risk of development of arteriovenous fistulae.
Circulation.
1985;
72
471-479
-
29
Conte S, Gewillig M, Eyskens B, Dumoulin M, Daenen W.
Management of late complications after classic Fontan procedure by conversion to total cavopulmonary connection.
Cardiovascular Surgery.
1999;
7
651-655
-
30
Shannon F, Campbell D.
Right atrial thrombosis: rare complication of the modified Fontan procedure.
Pediatr Cardiol.
1986;
7
209-212
-
31
Leung M, Benson L, Smallhorn J, Williams W, Trusler G, Freedom R.
Abnormal cardiac signs after Fontan type of operation: indicators of residua and sequelae.
Br Heart J.
1989;
61
52-58
-
32
Girod D, Fontan F, Deville C, Ottenkamp J, Choussat A.
Long-term results after the Fontan operation for tricuspid atresia.
Circulation.
1987;
75
605-610
-
33
Lemmer J, Coran A, Behrendt D, Heidelberger K, Stern A.
Liver fibrosis (cardiac cirrhosis) five years after modified Fontan operation for tricuspid atresia.
J Thorac Cardiovase Surg.
1983;
86
757-760
-
34
Matsuda E, Covino E, Hirose H, Nakano S, Kishimoto K, Miyamoto Y, Nishigaki K, Takano H, Ohtake S, Sakaki S, Ogawa M, Taenaka N, Kawashima Y.
Acute liver dysfunction after modified Fontan operation for complex cardiac lesions.
J Thorac Cardiovasc Surg.
1988;
96
219-226
-
35
Ilbawi M, Idress F, Muster A, DeLeon S, Berry T, Duffy E, Paul M.
Effects of elevated coronary sinus pressure on left ventricular function after the Fontan operation.
J Thorac Cardiovasc Surg.
1986;
92
231-237
-
36
Ward K, Fisher D, Michael L.
Elevated coronary sinus pressure does not alter myocardial blood flow or left ventricular contractile function in mature sheep. Implications after the Fontan operation.
J Thorac Cardiovasc Surg.
1988;
95
511-515
-
37
Denef B, Dumoulin M, Van der Hauwaert L G.
Usefulness of echocardiographic assessment of right ventricular and pulmonary trunk size for estimating magnitude of left-to-right shunt in children with atrial septal defect.
Am J Cardiol.
1985;
55
1571-1575
-
38 Rudolph A M. The fetal circulation. In: Congenital diseases of the heart. Chicago; Year Book Publishers 1974: 1-16
-
39
Hurwitz R A, Caldwell R L, Girod D A, Hurwitz R A, Caldwell R L, Girod D A, Wellman H.
Left ventricular function in tricuspid atresia: a radionuclide study.
J Am Coll Cardiol.
1986;
8
916-921
-
40
Riordan C J, Randsbaek F, Storey J H, Montgomery W D, Santamore W P, Austin E H.
Balancing pulmonary and systemic arterial flows in parallel circulations: the value of monitoring systemic venous oxygen saturations.
Card Young.
1997;
7
74-79
-
41
Park M K.
Pathophysiology of cyanotic congenital heart disease. In Pediatric Cardiology for Practitioners.
St louis, Mosby.
1996;
3rd ed
114-127
-
42
Hijazi Z M, Fahey J T, Kleinman C S, Kopt G S, Hellenbrand W E.
Hemodynamic evaluation before and after closure of fenestrated Fontan.
Circulation.
1992;
86
196-202
-
43 Lawrenson J, Gewillig M. The ventricle in the functionally univentricular heart. In: Anderson R The right heart in congenital heart disease.
-
44
Gewillig M, Lundström U, Deanfield J, Bull C, Franklin R, Graham T, Wyse R.
Impact of the Fontan operation on left ventricular size and contractility in tricuspid atresia.
Circulation.
1990;
81
118-127
-
45
Gewillig M, Deanen W, Aubert A, Van der Hauwaert L.
Abolishment of a chronic volume overload: implications for diastolic function of the systemic ventricle immediately after Fontan repair.
Circulation.
1992;
56
93-99
-
46
Silverstein D M, Hansen D P, Ojiambo H P, Griswold H E.
Left ventricular function in severe pure mitral stenosis as seen at the Kenyatta National Hospital.
Br Heart J.
1980;
99
727-733
MD PhD Professor Marc Gewillig
Paediatric Cardiology University Hospital Gasthuisberg
3000 Leuven
Belgium
Phone: +32 16 343865
Fax: +32 16 343981
Email: marc.gewillig@uz.kuleuven.ac.be
