105
Partial "Anomalous" Hepatic Venous Connection Associated with Secundum Atrial Septal Defect M. K. Heinemann . K. 1. Oldhafer, and G. Ziemer Division of Thoracic and"cardiovascular Surgery. Surgical Center, Hannover Medical School,Hannover, Germany
Postnatal connection of hepatic veins with the coronary sinus represents the rar e persistence of a normal early embryonic development. A case is presented where this was found intraoperatively, associated with a secundum type atrial septal defect. Knowledge of this anatomic variation and its specific featur es may facilitate preoperative diagnosis, thereby preventing surprises in the operating room. Keywords
Leberveneneinmiindung in den Koronarsinus bei Vorhofseptumdefekt (ASD II) Bei der postnata len Einmiindung von Lebervenen in den Koronarsinus hand elt es sich urn die seltene Persistenz einer friihen embryonalen Entwicklung. Es wird ein Fall geschildert, bei dem diese .An omalie" in Verbindung mit einem Vorhofseptumdefekt vom Secundumtyp (ASD II) intraoperativ entdeckt wurde. Das Wissen urn diese ana tomische Variante und ihre besonderen Merkmale erleichtert die priiopera tive Diagnose und hilft, Oberraschungen im Operationssaa l zu verhindern .
Anomalous systemic venous return - Hepati c veins - Anomalous hepatic venous connection - Atrial septal defect
Introduction "Anoma lous" hep atic veno us connection is a rar e finding in pati ent s witho ut heterotaxy. Ifunsuspected pri or to surgery, as in the case of thi s report. it ma y cre ate a puzzling situation for th e surgeon who is faced with un expected venous return into the right atrium, even after occlusion of both venae cavae and the aorta being cross-clamped. Current echocardiographic techn iques sh ould be able to diagnose unu su al variations in systemi c veno us connections pr eop eratively.
clamping. No persist ent left superior vena cava was found . Inspection of th e coronary sinus revealed an additional vas cular orifice at its bott om, 1 ern from th e ostium. Luxati on of th e heart showe d a vessel 0.7 to 1.0 cm in diam eter , pen etratin g the diaphragm and connecting with th e CS (Fig. 1). Whe n this vesse l was pr obed , it continued ben eath th e diaphragm into th e liver.
Case Report A 35-year- old Afghan mal e had progressive dyspnea on exertion and an episode of fainting. On examination there was no cyanosis; blood pr essure was 120/ S0 mmHg, puls e rate SO/ min . A harsh systolic murmur with fixed splitting of the second heart sound could be heard at the left stern al border . Chest radiograph sh owed an enlarge d heart with incre as ed pulmonary markings . Echoca rdiography and cardiac cathe rizat ion confirme d th e dia gnosis of a large secundum type atrial sept al defect (ASD Il), Th e ope ration wa s performed on hypoth ermic cardiopulmonary bypass with cardioplegic arres t. Both venae cavae we re cannulated. Via a right atriotomy a 3 by 2 cm ASD II (stretched foramen ovale type) was visualized. The ope ning of th e coronary sinus (CS) was 1.5 em in diam eter and showed significant blood return desp ite aortic cross-
Thorac. cardiovasc. Surgeon 40 (1992) 105-107 © GeorgThieme Verlag Stuttgart · NewYork
Fig. 1 Surgeon's view.The headofthepatient is totheleft.The heart is lifted bythe surgeon'sfingers (F). Hepaticvein (HV) coming fromthe diaphragm(D) andconnectingto the coronary sinus(CS). Ive = cannulated inferiorvenacava
Received for Publication: September 23, 1991
Downloaded by: NYU. Copyrighted material.
Summary
Thom e. eardiovase. Slirgeon40 (1992)
Fig.2 Coronary angiography (venousphase): funnel-shaped coronarysinus (CS). Hepatic vein (HV) filledretrogradely, comingfrom below
In order to stop th e venous backflow, th e ab errant vessel was clamped . Th e ASD wa s closed with a patch of autologous peri cardium , pre-treated with 0.6 % gluta ra ldeh yd e. Th e clamp on th e ab errant vess el wa s th en released and venous backflow started imm edi at elv. Whilst th e atriotomy was clos ed , th e snares on th e ve nae cavae wer e releas ed . After a de qua te deairing, the ao rtic cro sscla m p was ope ne d and th e ope r atio n was contin ued in th e usu al fashion. Th e patient's postoperati ve course was un eventful apart from temporary atrial fibrilla tion . Postoperative a bdom ina l son ogr aphy sh owed visce ra l situs solitus, a normal liver with ortho topic inferi or vena cava (lYC) and portal vein . Th e hepatic veins could not be visua lize d. Th e spl een appeared normal. Echoc a rdiogra phy showed com plete closure of th e ASD and a marked turbulence at the osti um of th e dilated CS. Re-evaluation of th e venous phase of the preoperative corona ry angiography showed a funnel-shaped CS with a dilatation just befor e its con ne ction with the righ t atri um . The ab er rant hepatic vein was ide nt ified, being filled retr ograd ely towards the diaph ragm (Fig. 2). Arte rial digital sub tra ctio n angiography revealed an accessory hepatic art ery from th e mes enteric artery. Th e hepatic veins gav e too little contrast to clearly visualize the "a bnorm al" vess el. In view of th e th erapeutic irrelevance of these findings postop er ati vely, we refr ained from furth er diagnostic pr ocedures. Dis cussion In th e ea rly em bryonic life (horizon XI = postovulati on age 22 -24 days) three pairs of veins drain int o th e left and right horn of th e sinus venosus: th e umbilical, th e comm on ca rdinal and th e vitellin e veins . Sub sequ ent oblite r ations and formations of vesse ls lead to th e development of th e inferi or and superior vena cava (lYC, SYC), of th e azyg os system , and of th e hepatic veins. Th e coro na ry sinus (CS)repres ents th e reduced left horn of th e sinus ve nos us and part of th e left comm on ca r dina l vein (8). Som e of th e ea rly embryo nic syst emic venous conne ctions with th e CS ma y persist postnatally. A persistent left superi or vena cava is th e most freq uent of th es e conn ection s in many normal hearts. The he patic veins (= the distal stu bs of th e om pha lomesenter ic veins) connect w ith bot h the righ t and
M. K. Heinemann. K. J. Oldhufer. and G. Ziemer
th e left horn of the sinus venosus. As th e liver shifts towards th e right and th e unpaired hepatic segm ent of th e IYC develops, all th e hepatic veins usually connec t with th e suprahepatic segm ent of th e IYC (8). In some case s , how ever, th e con ne ction of th e left hepatic veins with th e left horn of th e sinus venosus ma y persist. As th e left anterior ca rdinal vein inv olut es (after th e development of th e inn ominate vein), th e pr oxim al part of th e left horn of th e sinus veno sus becom es th e CS. Wh en th e left hep ati c veins maintain th eir connec tion with th e left horn of th e sin us veno sus, th ey will obviously drain into the CS postnatally. In visce r al het erotaxy (asplenia or polyspl enia) th ere exists abnormal symme try of th e liver, th e syst emic veins, th e lung lobation an d th e atrial appendag es . Abnormalities of systemic venous return and espe cia lly th e hepatic veins are frequent and repres ent persistence of the ea rly em bryoni c systemic veno us connections (0) . There a re va rio us nu m bers and patterns of hep a tic veins postn a tally. Generally, three m ai n hep at ic veins d rai n into th e IYC below the dia phragm . In most cases th e r igh t hepatic vein dra ins separately, whil e th e middle and left hepatic vein form a common trunk . Accessory left or right hepatic veins dra ining into th e IYC may occur. In addition to th es e ma in veins, an ind efinite number of inferior dorsal veins ma y connec t directly to th e IYC (7). Partial "Anom alous" Hepatic Yen ous Connection (PAHYC) ha s been rep orted in reviews of th oracic ve no us abnormalities (5), of an om alou s sys te mic ve no us connec tions 0 , 2), and of abnormaliti es of th e coro nary sinus cir culation (3, 6). Its association with ASD ha s also been des cribed (2, 3, 9). Th e abnormalit y of PAHYCdraining in to th e CS was first precisely identifi ed in vivo by Sanders using 2-D echoc a rdiogra phy (9). It was as sociated with a n ASD II and a per sistent du ctus a rte riosus in a newborn . LePere and associates des cribed two case s of a n anomalous inferior syst emic vein draining into CS (5) . In Montini 's classification of congenital anomalies involving th e CS, PAHYC is present in his subgroup I A: enla rge me nt of th e CS (=1) wit ho ut left-to-right shunt into CS (=A) (6). He mentions a desc r iption of this anomaly by Nabarro in 1903 . Reviewing ano malous veno us con nectio ns in heter otaxy, Van Praagh et al. repor ted 2 cas es wit h a hep at ic vein ente ring the right atrium via a normal CS separately from th e IYC (0) . One case had asplenia , th e oth er polysp len ia . Th e surgeon should alwa ys suspect anomalous systemi c venous connection with th e CS wh en its orific e is found to be enla rged. Wh en excessive venous backflow int o th e right atrium occurs on total ca rdiopulmona ry byp as s and a left superi or vena cava ha s been rul ed out, "ano malous" con ne ction of some of th e hepatic veins is the m ost likely possibility. Th es e vess els ca n be ca nn ula te d se pa rat ely or temporarily clamped , dep ending up on th e duration of th e op eration planned . Ligation of su ch veins is inappropriate and can be dangerous. Th e same is true for a persistent left superior vena cava (4) or th e azygo s continuation of an int errupted IYC (5). Ackno wle dgeme n t Th e invaluabl e help a nd contributio ns by Stella Van Praa gh, M. D.. Childre n's Hosp ital, Bost on , USA. a re gra tefully ac knowledge d.
Downloaded by: NYU. Copyrighted material.
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Partial ·Anomalous · He patic Venous Connection A ssociate d with S ecundum Atrial S ep tal Defect Thom c. cardiovasc. Surgeon 40 (1992)
References
2
3
4
5 6
7
Crenshaw. R.. J. E. Okies. S. J. Phillips . L. I. Bonchek . and A. S tarr: Partial anomalous syst emic venous return : Report of surgical treat men t in two cases. J. Thora c. Cardiovasc. Surg. 69 (1975) 433-436 de Leoal. M.. D. G. Ritter. D. McGoon. and G. K. Danielson:Anomalous syst emic veno us connect ion. Sur gical considera tions . Mayo Clin. Proc. 50 (197 5) 599- 610 Frank. C. G.. and J. V. Ma loney : Surgical significance of congenital anomalies of the coronary sinus. J . Card iovasc. Surg. 9 (1968 ) 420-427 Freed. M. D.• A. Ros entha l. and W. F. Bernhard: Balloon occlusion of a per sisten t left su perio r vena cava in the preoperative evalua tion of syste mic venous return. J. Tho ra c. Cardiovasc . Surg. 65 (1973) 835 -839 LePere. R. H.. C. M. Kohler. P. Klinger. and J. Lowry: Intrath oraci c venous anoma lies . J. Thorac. Cardiovasc. Surg . 49 (1965 ) 599-6 13 Mantin i, E.• C. M. Grondin. C. W. Lilleh ei, and J. E. Edwards: Congenital an omalies involving the corona ry sinus. Circulatio n 33 (1966) 317 - 327 Nakamura. S.. and T. Tsuzuki: Surg ical anato my of the hepat ic
8 9
10
veins and the inferior vena cava. Surg. Gyneco!. Obstet. 152 (1981) 43-50 Patt en. B. M.: Human Embryology, 2nd ed. New York-Toronto-London : Me Graw-Hi1l1953 . pp. 643-646 S anders. S. P.: Anomalous hepat ic venou s connection to the coronary sinus diagnosed by two-dimensio nal echocardiogra phy. Am. J . Cardio!' 54 (1984) 458-459 Vall Praagh. S.. 1. Kreutzer. L. A lday. and R. Van Praagh: System ic and pulmona ry venous connections in visceral heter otaxy, with emphasis on th e diagnosis of th e atrial situs: a study of 109 postmortem cases . In: Clark, E. B., A. Taka o (eds.l: Developm enta l cardiology: mor phogenesis and function. Mount Kisco, NY:Futura Publishing Co 1990 . pp. 671- 727
Dr. Markus Heinemann clo The Children's Hospital Cardiac Surg ery 300 Longwood Avenue Boston. MA02 115 USA
Downloaded by: NYU. Copyrighted material.
1
107
Partial "Anomalous" Hepatic Venous Connection Associated with Secundum Atrial Septal Defect M. K. Heinemann . K. 1. Oldhafer, and G. Ziemer Division of Thoracic and"cardiovascular Surgery. Surgical Center, Hannover Medical School,Hannover, Germany
Postnatal connection of hepatic veins with the coronary sinus represents the rar e persistence of a normal early embryonic development. A case is presented where this was found intraoperatively, associated with a secundum type atrial septal defect. Knowledge of this anatomic variation and its specific featur es may facilitate preoperative diagnosis, thereby preventing surprises in the operating room. Keywords
Leberveneneinmiindung in den Koronarsinus bei Vorhofseptumdefekt (ASD II) Bei der postnata len Einmiindung von Lebervenen in den Koronarsinus hand elt es sich urn die seltene Persistenz einer friihen embryonalen Entwicklung. Es wird ein Fall geschildert, bei dem diese .An omalie" in Verbindung mit einem Vorhofseptumdefekt vom Secundumtyp (ASD II) intraoperativ entdeckt wurde. Das Wissen urn diese ana tomische Variante und ihre besonderen Merkmale erleichtert die priiopera tive Diagnose und hilft, Oberraschungen im Operationssaa l zu verhindern .
Anomalous systemic venous return - Hepati c veins - Anomalous hepatic venous connection - Atrial septal defect
Introduction "Anoma lous" hep atic veno us connection is a rar e finding in pati ent s witho ut heterotaxy. Ifunsuspected pri or to surgery, as in the case of thi s report. it ma y cre ate a puzzling situation for th e surgeon who is faced with un expected venous return into the right atrium, even after occlusion of both venae cavae and the aorta being cross-clamped. Current echocardiographic techn iques sh ould be able to diagnose unu su al variations in systemi c veno us connections pr eop eratively.
clamping. No persist ent left superior vena cava was found . Inspection of th e coronary sinus revealed an additional vas cular orifice at its bott om, 1 ern from th e ostium. Luxati on of th e heart showe d a vessel 0.7 to 1.0 cm in diam eter , pen etratin g the diaphragm and connecting with th e CS (Fig. 1). Whe n this vesse l was pr obed , it continued ben eath th e diaphragm into th e liver.
Case Report A 35-year- old Afghan mal e had progressive dyspnea on exertion and an episode of fainting. On examination there was no cyanosis; blood pr essure was 120/ S0 mmHg, puls e rate SO/ min . A harsh systolic murmur with fixed splitting of the second heart sound could be heard at the left stern al border . Chest radiograph sh owed an enlarge d heart with incre as ed pulmonary markings . Echoca rdiography and cardiac cathe rizat ion confirme d th e dia gnosis of a large secundum type atrial sept al defect (ASD Il), Th e ope ration wa s performed on hypoth ermic cardiopulmonary bypass with cardioplegic arres t. Both venae cavae we re cannulated. Via a right atriotomy a 3 by 2 cm ASD II (stretched foramen ovale type) was visualized. The ope ning of th e coronary sinus (CS) was 1.5 em in diam eter and showed significant blood return desp ite aortic cross-
Thorac. cardiovasc. Surgeon 40 (1992) 105-107 © GeorgThieme Verlag Stuttgart · NewYork
Fig. 1 Surgeon's view.The headofthepatient is totheleft.The heart is lifted bythe surgeon'sfingers (F). Hepaticvein (HV) coming fromthe diaphragm(D) andconnectingto the coronary sinus(CS). Ive = cannulated inferiorvenacava
Received for Publication: September 23, 1991
Downloaded by: NYU. Copyrighted material.
Summary
Thom e. eardiovase. Slirgeon40 (1992)
Fig.2 Coronary angiography (venousphase): funnel-shaped coronarysinus (CS). Hepatic vein (HV) filledretrogradely, comingfrom below
In order to stop th e venous backflow, th e ab errant vessel was clamped . Th e ASD wa s closed with a patch of autologous peri cardium , pre-treated with 0.6 % gluta ra ldeh yd e. Th e clamp on th e ab errant vess el wa s th en released and venous backflow started imm edi at elv. Whilst th e atriotomy was clos ed , th e snares on th e ve nae cavae wer e releas ed . After a de qua te deairing, the ao rtic cro sscla m p was ope ne d and th e ope r atio n was contin ued in th e usu al fashion. Th e patient's postoperati ve course was un eventful apart from temporary atrial fibrilla tion . Postoperative a bdom ina l son ogr aphy sh owed visce ra l situs solitus, a normal liver with ortho topic inferi or vena cava (lYC) and portal vein . Th e hepatic veins could not be visua lize d. Th e spl een appeared normal. Echoc a rdiogra phy showed com plete closure of th e ASD and a marked turbulence at the osti um of th e dilated CS. Re-evaluation of th e venous phase of the preoperative corona ry angiography showed a funnel-shaped CS with a dilatation just befor e its con ne ction with the righ t atri um . The ab er rant hepatic vein was ide nt ified, being filled retr ograd ely towards the diaph ragm (Fig. 2). Arte rial digital sub tra ctio n angiography revealed an accessory hepatic art ery from th e mes enteric artery. Th e hepatic veins gav e too little contrast to clearly visualize the "a bnorm al" vess el. In view of th e th erapeutic irrelevance of these findings postop er ati vely, we refr ained from furth er diagnostic pr ocedures. Dis cussion In th e ea rly em bryonic life (horizon XI = postovulati on age 22 -24 days) three pairs of veins drain int o th e left and right horn of th e sinus venosus: th e umbilical, th e comm on ca rdinal and th e vitellin e veins . Sub sequ ent oblite r ations and formations of vesse ls lead to th e development of th e inferi or and superior vena cava (lYC, SYC), of th e azyg os system , and of th e hepatic veins. Th e coro na ry sinus (CS)repres ents th e reduced left horn of th e sinus ve nos us and part of th e left comm on ca r dina l vein (8). Som e of th e ea rly embryo nic syst emic venous conne ctions with th e CS ma y persist postnatally. A persistent left superi or vena cava is th e most freq uent of th es e conn ection s in many normal hearts. The he patic veins (= the distal stu bs of th e om pha lomesenter ic veins) connect w ith bot h the righ t and
M. K. Heinemann. K. J. Oldhufer. and G. Ziemer
th e left horn of the sinus venosus. As th e liver shifts towards th e right and th e unpaired hepatic segm ent of th e IYC develops, all th e hepatic veins usually connec t with th e suprahepatic segm ent of th e IYC (8). In some case s , how ever, th e con ne ction of th e left hepatic veins with th e left horn of th e sinus venosus ma y persist. As th e left anterior ca rdinal vein inv olut es (after th e development of th e inn ominate vein), th e pr oxim al part of th e left horn of th e sinus veno sus becom es th e CS. Wh en th e left hep ati c veins maintain th eir connec tion with th e left horn of th e sin us veno sus, th ey will obviously drain into the CS postnatally. In visce r al het erotaxy (asplenia or polyspl enia) th ere exists abnormal symme try of th e liver, th e syst emic veins, th e lung lobation an d th e atrial appendag es . Abnormalities of systemic venous return and espe cia lly th e hepatic veins are frequent and repres ent persistence of the ea rly em bryoni c systemic veno us connections (0) . There a re va rio us nu m bers and patterns of hep a tic veins postn a tally. Generally, three m ai n hep at ic veins d rai n into th e IYC below the dia phragm . In most cases th e r igh t hepatic vein dra ins separately, whil e th e middle and left hepatic vein form a common trunk . Accessory left or right hepatic veins dra ining into th e IYC may occur. In addition to th es e ma in veins, an ind efinite number of inferior dorsal veins ma y connec t directly to th e IYC (7). Partial "Anom alous" Hepatic Yen ous Connection (PAHYC) ha s been rep orted in reviews of th oracic ve no us abnormalities (5), of an om alou s sys te mic ve no us connec tions 0 , 2), and of abnormaliti es of th e coro nary sinus cir culation (3, 6). Its association with ASD ha s also been des cribed (2, 3, 9). Th e abnormalit y of PAHYCdraining in to th e CS was first precisely identifi ed in vivo by Sanders using 2-D echoc a rdiogra phy (9). It was as sociated with a n ASD II and a per sistent du ctus a rte riosus in a newborn . LePere and associates des cribed two case s of a n anomalous inferior syst emic vein draining into CS (5) . In Montini 's classification of congenital anomalies involving th e CS, PAHYC is present in his subgroup I A: enla rge me nt of th e CS (=1) wit ho ut left-to-right shunt into CS (=A) (6). He mentions a desc r iption of this anomaly by Nabarro in 1903 . Reviewing ano malous veno us con nectio ns in heter otaxy, Van Praagh et al. repor ted 2 cas es wit h a hep at ic vein ente ring the right atrium via a normal CS separately from th e IYC (0) . One case had asplenia , th e oth er polysp len ia . Th e surgeon should alwa ys suspect anomalous systemi c venous connection with th e CS wh en its orific e is found to be enla rged. Wh en excessive venous backflow int o th e right atrium occurs on total ca rdiopulmona ry byp as s and a left superi or vena cava ha s been rul ed out, "ano malous" con ne ction of some of th e hepatic veins is the m ost likely possibility. Th es e vess els ca n be ca nn ula te d se pa rat ely or temporarily clamped , dep ending up on th e duration of th e op eration planned . Ligation of su ch veins is inappropriate and can be dangerous. Th e same is true for a persistent left superior vena cava (4) or th e azygo s continuation of an int errupted IYC (5). Ackno wle dgeme n t Th e invaluabl e help a nd contributio ns by Stella Van Praa gh, M. D.. Childre n's Hosp ital, Bost on , USA. a re gra tefully ac knowledge d.
Downloaded by: NYU. Copyrighted material.
106
Partial ·Anomalous · He patic Venous Connection A ssociate d with S ecundum Atrial S ep tal Defect Thom c. cardiovasc. Surgeon 40 (1992)
References
2
3
4
5 6
7
Crenshaw. R.. J. E. Okies. S. J. Phillips . L. I. Bonchek . and A. S tarr: Partial anomalous syst emic venous return : Report of surgical treat men t in two cases. J. Thora c. Cardiovasc. Surg. 69 (1975) 433-436 de Leoal. M.. D. G. Ritter. D. McGoon. and G. K. Danielson:Anomalous syst emic veno us connect ion. Sur gical considera tions . Mayo Clin. Proc. 50 (197 5) 599- 610 Frank. C. G.. and J. V. Ma loney : Surgical significance of congenital anomalies of the coronary sinus. J . Card iovasc. Surg. 9 (1968 ) 420-427 Freed. M. D.• A. Ros entha l. and W. F. Bernhard: Balloon occlusion of a per sisten t left su perio r vena cava in the preoperative evalua tion of syste mic venous return. J. Tho ra c. Cardiovasc . Surg. 65 (1973) 835 -839 LePere. R. H.. C. M. Kohler. P. Klinger. and J. Lowry: Intrath oraci c venous anoma lies . J. Thorac. Cardiovasc. Surg . 49 (1965 ) 599-6 13 Mantin i, E.• C. M. Grondin. C. W. Lilleh ei, and J. E. Edwards: Congenital an omalies involving the corona ry sinus. Circulatio n 33 (1966) 317 - 327 Nakamura. S.. and T. Tsuzuki: Surg ical anato my of the hepat ic
8 9
10
veins and the inferior vena cava. Surg. Gyneco!. Obstet. 152 (1981) 43-50 Patt en. B. M.: Human Embryology, 2nd ed. New York-Toronto-London : Me Graw-Hi1l1953 . pp. 643-646 S anders. S. P.: Anomalous hepat ic venou s connection to the coronary sinus diagnosed by two-dimensio nal echocardiogra phy. Am. J . Cardio!' 54 (1984) 458-459 Vall Praagh. S.. 1. Kreutzer. L. A lday. and R. Van Praagh: System ic and pulmona ry venous connections in visceral heter otaxy, with emphasis on th e diagnosis of th e atrial situs: a study of 109 postmortem cases . In: Clark, E. B., A. Taka o (eds.l: Developm enta l cardiology: mor phogenesis and function. Mount Kisco, NY:Futura Publishing Co 1990 . pp. 671- 727
Dr. Markus Heinemann clo The Children's Hospital Cardiac Surg ery 300 Longwood Avenue Boston. MA02 115 USA
Downloaded by: NYU. Copyrighted material.
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