Pathophysiological Aspects after Cavopulmonary Anastomosis

 

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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.

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
  CrossrefPubMedGoogle Scholar
• 2 Rudolph A M. Congenital Heart Disease. Chicago; Year Book Medical Publishers 1974: 124-126
  Google Scholar
• 3 Dick M, Fyler D C, Nadas A S. Tricuspid Atresia: clinical course in 101 patients.  Am J Cardiol. 1975;  36 327-337
  CrossrefPubMedGoogle Scholar
• 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
  Google Scholar
• 5 Fontan F, Baudet E. Surgical repair of tricuspid atresia.  Thorax. 1971;  26 240-248
  CrossrefPubMedGoogle Scholar
• 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
  Google Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 10 Gale A, Danielson G, McGoon D, Wallace R, Mair D. Fontan procedure for tricuspid atresia.  Circulation. 1980;  62 91-96
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 16 Kreutzer G. Atriopulmonary anastomosis.  J Thorac Cardiovasc Surg. 1982;  83 427-436
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 26 Glenn W. Superior vena cava-pulmonary artery anastomosis.  Ann Thorac Surg. 1984;  37 9-15
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 30 Shannon F, Campbell D. Right atrial thrombosis: rare complication of the modified Fontan procedure.  Pediatr Cardiol. 1986;  7 209-212
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 38 Rudolph A M. The fetal circulation. In: Congenital diseases of the heart. Chicago; Year Book Publishers 1974: 1-16
  Google Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 41 Park M K. Pathophysiology of cyanotic congenital heart disease. In Pediatric Cardiology for Practitioners.  St louis, Mosby. 1996;  3rd ed 114-127
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 43 Lawrenson J, Gewillig M. The ventricle in the functionally univentricular heart. In: Anderson R The right heart in congenital heart disease. 
  Google Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar

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