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Transumbilical balloon atrial septostomy for transposition of the great arteries in infants under the age of 60 hours
January.
174
Brief
Communications
Case2. D.F., a 29-year-old man with rheumatic mitral valve disease,underwent mitral valve replacement with a No. 33 Carpentier-Edwards porcine valve. The postoperative course was uneventful. An ME performed before discharge showed mild enlargement of the LV (enddiastolic diameter of 5.9 cm) and paradoxical motion of the IVS (Fig. 2, A). Four weeksfollowing the operation the patient wasreadmitted for severedyspnea.Blood pressure was 90/50 mm Hg, heart rate was 128/min, and respirations were 29/min. He was lethargic, confused, and had rales over the entire lung fields. There were no murmurs. The ECG revealed sinus tachycardia. A repeat ME showed: (1) LV diameters and 1% paradoxical motion unchanged from previous examination; (2) diastolic fluttering of the posterior porcine leaflet; and (3) early diastolic anterior hump of the stents (Fig. 2, R). LV cineangiographyrevealed 4+ mitral regurgitation. During surgery the mitral bioprosthesis was found detached about 50 degreesaround the ring. The mitral valve was replaced, and the tricuspid valve, becauseof significant regurgitation, wasrepaired with a Carpentier anulus.The patient died after surgery becauseof persistent low output state. The most common ME finding reported in mitral prosthesisdetachment is LV volume overload with early postoperative normalization of septal motion4 However, this, is an indirect and nonspecificsign, since it can occur with a leaking mitral or aortic prosthesis,4and with both paraprosthetic and intraprosthetic regurgitation.? Furthermore, it is not always present, as for instance when right ventricular diastolic overload coexists, as in our second patient with significant tricuspid regurgitation. Another sign reported with dehiscent porcine mitral valve has been diastolic fluttering of porcine leaflets.? This finding is alsononspecific; in fact, it has been reported in native aortic valve regurgitation and in patients with torn porcine mitral leaflets.’ The sign we found here is a new one. Normally the bioprosthetic stent moves anteriorly during systole and posteriorly at the beginning of diastole (Figs. 1, A and 2, A). Conversely, in our patients the initial diastolic posterior movement was substituted by an early diastolic anterior hump (Figs. 1, B and 2, B). Probably this finding is the equivalent of the early diastolic hump describedby others”.” in casesof paravalvular leaking mechanicalprostheses.For this reason,and sinceit has not been reported in patients with other kinds of porcine mitral valve dysfunction, the early diastolic hump highly suggestsa dehiscent mitral bioprosthesis. Furthermore, since prosthetic mitral paravalvular regurgitation may be present without murmurs making clinical recognition difficult, ME may be an important diagnostictool for detecting this complication. REFERENCES
I. Alam M, Madrazo and two-dimensional valve dysfunction. 2. Salati A, Narducci
AC, Magilligan DJ, Goldstein S: M-mode echocardiographic features of porcine Am J Cardiol 432502, 1979. C, Pino PG, Chidichimo G, Jacovella G:
American
Heart
1984 Journal
Patologia delle protesi mitraliche porcine. Rilievi ecocardiografici in cinque casi con rigurgito intra-para-protesico. G Ital Cardiol 10:605, 1980. Horowitz MS, Goodman DJ, Hancock EW, Popp RL: Noninvasive diagnosis of complications of the mitral bioprosthesis. J Thorac Cardiovasc Surg 71:450, 1976. Feigenbaum H: Echocardiography. 3rd ed. Philadelphia, 1981, Lea & Febiger, p 313. Yuste P, Aza V, Minguez I, Asin Cardiel E, Martinez Bordiu C: Echocardiographic evaluation of mitral valve prosthesis malfunction. In Kazner E, editor: Ultrasonics in medicine. Amsterdam, 1975, Excerpta Medica, p 239. Bernal-Ramirez JA, Phillips JH: Echocardiographic study of malfunction of the Bjork-Shiley prosthetic heart valve in the mitral position. Am .J Cardiol 40:449, 1977.
Transumbilical balloon atrial septostomy for transposition of the great arteries in infants under the age of 60 hours N. Roguin, P. Sujov, J. Montag, M. Zeltzer, and E. Riss. Haifa and Nahariya, Israel Balloon atria1 septostomy (BAS)’ has dramatically improved life expectancy for infants with complete transposition of the great arteries (TGA). It is the purpose of this report to analyze our results with the transumbilical BAS approach and consider the optimal age to use the umbilical vein technique in babies with TGA. Ten patients below the age of 60 hours with complete TGA underwent cardiac catheterization during the period from February 1,1979, to August 30,1981, at the Rambam Medical Center, in Haifa, Israel. In the caseof 3 of the 10 patients, it was impossibleto perform the BAS through the umbilical vein route, and a saphenofemoraljunction cutdown technique wasemployed. The agesof the infants at the time of the procedure ranged from 16 to 56 hours (mean31 hours), the weight being from 2.2 to 3.8 kg (mean 3.2 kg). All the babieswere examined without sedation or anesthesia.In the three patients mentioned above, local anesthesiahad to be given prior to cutdown, the agesof these babies at the time being 16, 48, and 56 hours. At first, a No. 5 angiographic catheter was introduced through the umbilical vein-ductus venosusto the heart. Pressurerecordings and blood samplesfor oxygen determinations were taken In casethe diagnosisof TGA was not clear enough, cineangiography was necessary-either in the right or left ventricle. After confirmation of the diagnosis, a No. 5 Miller single-lumen balloon atria1 septostomy catheter (Edwards Laboratories, Santa Ana, From the Departments of Cardiology and Neonatology, Center, and Faculty of Medicine, Technion-Institute the Department of Neonatology, Nahariya Government Reprint Center,
requests: N. Roguin. Haifa, Israel.
M.D.,
Dept.
of Cardiology,
Rambam Medical of Technology: and Hospital. Rambam
Medical
Volume
107
Number
I
California) was introduced through the umbilical veinductus venosuswithout difficulty in the left atrium. The balloon wasthen inflated with 3 to 3.5 cc of 20% solution of contrast medium, and was rapidly jerked back to the inferior vena cava. Immediately after withdrawal it was advanced to the right atrium and then deflated rapidly. During this procedure, one hand was pressedgently over the umbilical area of the baby to control the withdrawal of the balloon catheter. This procedure was repeated from five to eight times. The catheter was then changed for a No. 5 catheter, and pressuresin the left and right atrium weretaken. A blood samplefor oxygen determinations was made in superior vena cava and/or aorta. During the entire process,a careful monitoring of blood gasesand temperature of the baby were done. All infants were treated with systemic antibiotics (penicillin and kanamytin) for 4 days as prophylaxis against sepsis.We carried out a complete investigation with cineangiography into the right and left ventricles and with an aortogram at the age of 3 or 4 months’; at the age of 6 months surgical intervention was necessary.During the entire period the babies were examined in our outpatient clinic twice a month; in addition to all the clinical signs we carefully controlled the level of plasma hemoglobin, and iron supplements were given when necessary. Out of eight patients examined under the age of 36 hours (mean 26.2 hours), the BAS was successfully performed in seven of them (87.5?;) through the transumbilical route without resorting to anesthesiaor premeditation. No perforation of the heart or sepsisor bleedingwere observed,while only one baby presentedabdominal distension,which persisted for 24 hours. After BAS there wasan increaseof the aortic or superior vena cava oxygen saturation of 17% mean, as well as an equalization of atria1 pressures.The babies all showedimproved clinical and metabolical condition, and were dischargedfrom the hospital 5 days after the procedure. Out of seven babies who underwent transumbilical BAS, five had simple TGA: one had TGA with pulmonic stenosisand one had TGA with large ventricular septal defect. One baby (TGA and pulmonic stenosis)died at the ageof 40 days from aspiration pneumonia.Six babieswere reinvestigated at the age of 4 months and underwent surgical correction at the age 6 months: four had a successfulbaffle procedure and two died-one with a simple TGA after an “anatomic” procedure, and the secondafter a baffle procedure and closureof the ventricular septal defect. In five out of the six babies,the atria1 septal defect was measured by the surgeon and ranged from 1.1to 14 mm in diameter, while in the patient who died at the ageof 40 days, the diameter of the atria1 septal defect was 12 mm. The ductus venosus constitutes a direct connection betweenthe umbilical vein and the inferior vena cava. It is about half the size of the umbilical vein,3 functioning asa low resistancebypassto the heart, and is unresponsiveto changesin the blood gasesor acid-baseequilibrium. It is, however, constricted by high concentration of epineph-
Brief
Communications
175
rine.4The postnatal closureof the ductus venosusappears to be a result of decreasein pressurein the portal sinus following cessation of flow in the umbilical vein and resulting in retraction and narrowing of its origin while assuminga slitlike appearance.The functional closure of the ductus venosusoccurs during the first minutes after birth and is not complete. The organic closure of the ductus venosusbeginsat its narrow origin, with proliferation of the obliteration tissue in specially structured border strips. The closure is completed between the fifteenth and twentieth day.3 Owing to the possibility of liver and portal vein infection, Rudolph4is not in favor of performing umbilical vein cardiac catheterization after the third day of life. He suggestsmaking a brief attempt to manipulate the catheter through the ductus venosus,and if this doesnot prove satisfactory, he prefers to dissectthe groin. We tend to sharehis view that there is little hope of successin the transumbilical approach, and therefore will not consider it after the third day of life. At the present time there are three technical possibilities of performing balloon septostomyin babieswith TGA: the femoral vein percutaneous technique, the saphenofemoral junction cutdown technique, and the transumbilical approach (in addition, there is also the possibility of using an axillary vein cutdown technique, making it more difficult, however, to perform BAS through this route). The percutaneoustechnique requires a No. 6 or 7 sheath for the femoral vein to introduce a balloon septostomy catheter. In capablehandsit is a goodtechnique, but often could unfortunately be traumatic for the baby, producing an inguinal hematoma. Should the femoral vein be posterior in relation to the femoral artery, it may be difficult or even impossibleto adopt this approach. Keane et a1.5reported an incidence of 16% of iliac vein or inferior vena cava thrombosiswhen this technique was used, and this complication was even two or three times higher in infants who underwent BAS than in those who did not. However, this was not of statistical significance owing to the relatively small number of patients involved. One factor suspectedof predisposingto inferior vena cava thrombosis is prolonged use of indwelling venous catheters; however, this factor was present in only 1 of the 12 patients reported.5Mathews et a1.6reported that the useof balloon catheters-either septostomy or flow-directedmay have contributed to venous thrombosis diagnosedin 19 (80%) of 22 patients. Venous thrombosis is a serious complication in patients with TGA requiring repetitive cardiac catheterization, and both the femoral veins will no longer be available. In addition to the technical points mentioned, other severecomplications have beenreported following inferior vena cava thrombosis, including pulmonary emboli and thrombosis and renal and adrenal veins. Technical skill is essentialin performing the saphenofemoral junction cutdown, as otherwise bleeding may result and repair of the vein after septostomy could produce someedemaand/or cyanosisof the leg. Skin infections are not uncommon, and sepsishas been noted,7 as well as thrombosis of the femoral vein or inferior vena cava.
176
Brief
Communications
However, the incidence of late complications is relatively low (l.2%).5 Porter et a1.7reported an experience with cardiac catheterization in the first week of life, and concluded that the umbilical vein route took 30 minutes lessthan either the percutaneousor cutdown techniques. We also feel that time is a factor of great importance in very ill babieswith TGA. To the best of our knowledge, the first transumbilical BAS in a baby 56 hours of agewas reported by Abinader et a1.8from our institution. The secondcase was reported by Romney et a1.3Newfeld et al.“’ reported 16 casesof transumbilical BAS in babies belowthe ageof 4 days; in 20% of casesit wasnot possible to use the umbilical approach. However, the authors did not correlate the age of the babies with failure of this method. No infection, bleeding, or liver damage was reported. We have undergonethe experienceof performing transumbilical BAS with successin 7 out of 10babiesbelow the ageof 60 hours, and in sevenout of eight cases(87.5%) in babiesbelow the ageof 36 hoursthe procedure wascarried out quickly and without undue complications. No anesthesia or premeditation was used. In treating babieswith suspectedTGA, we do not recommend introducing any umbilical vein or artery catheter before the procedure in order to avoid manipulation and possibilities of previous hemorrageand infection in the umbilical area. In conclusion,we recommendtransumbilical BAS as the method of choicefor babieswith TGA, especiallythose under the age of 36 hourswhen the rate of successis approximately 90%. The procedure is safe, and can be performed easily and quickly, leaving both saphenousveins available for future recatheterization. This study stressesthe need for early treatment of patients suspectedof having TGA.
REFERENCES 1. Rashkind 2.
3. 4. 5.
6.
7.
8. 9.
19.
WJ, Miller WW: Creation of an atria1 septal defect without thoractomy. JAMA 196:991, 1966. Leenage R, Aquetti A, Graham G, Taylor J, Macartney FJ: Factors influencing survival after balloon atria1 septostomy for complete transposition of great arteries. Br Heart J 15:559, 1981. Lind J: Human fetal and neonatal circulation. Some structural and functional aspects. Eur J. Cardiol 5(3):265, 1977. Rudoloh AM: Conaenital diseases of the heart. Chicago,. 1974, Year Book Medical Publishers, Inc, p 18. Keane JF, Lang P, Newburger J, Fyler DC: Iliac vein-inferior caval thrombosis after cardiac catheterization in infancy. Pediatr Cardiol 1:257, 1980. Mathews RA, Park SC, Neches WH, Fricker FJ, Lenox CC, Zuberbuhler JR: Iliac venous thrombosis in infants and children after cardiac catheterization. Cathet Cardiovasc 5:67, 1979. Porter CJ, Gillete PC, Mullins CE, McNamara DC: Cardiac catheterization in the neonate. A comparison of three techniques. J Pediatr 93(1):97, 1978. Abinader E, Zeltzer M, Riss E: Transumbilical atria1 septostomy in the newborn. Am J Dis Child 119:354, 1970. Romney D, Katzuni E, Aygen MM: Transumbilical balloon atria1 septostomy in a case of transposition of the great vessels with situs inversus totalis. Isr J Med Sci 8:529, 1972. Newfeld EA, Purcell C, Paul MH, Cole RV, Muster AJ: Transumbilical balloon atria1 septostomy in 16 infants with transposition of the great arteries. Pediatrics 54:495, 1974.
American
Familial hypertrophic preexcitation
cardiomyopathy
January, 1994 Heart Journal
and
Andrew M. Hauser, M.D., Seymour Gordon, M.D., and Gerald C. Timmis, M.D. Royal Oak, Mich. The familial occurrence of hypertrophic cardiomyopathy (HCM) has been noted in numerous reviews,‘,2 and an autosomal dominant mode of inheritance with variable expressivity and penetrance has been proposed.‘,” The familial occurrence of Wolff-Parkinson-White (WPW) conduction is rare,4 an autosomal dominant mode of inheritance alsohaving been suggested.“,”Although HCM is at times associated with WPW conduction,2,“-7the coexistence of HCM and WPW conduction in multiple family membersof the samegeneration hasbeen reported only twice.“,” We now report a third family in which two generations manifested coexisting WPW conduction and HCM of severe and diffuse nature. Case 1. The mother of the two afflicted children described below presented at age 26 years with a 3-year history of progressive dyspnea and cardiomegaly. Her ECG (Fig. 1) demonstrated morphologic features of type B WPW conduction. Progressive heart failure led to her death 1 year after presentation. Available autopsy records reported severe diffuse cardiac hypertrophy and normal coronary arteries. The patient bore two sons;cases2 and 3 below. Case 2. Becauseof an asymptomatic systolic murmur and cardiomegaly on chest x-ray examination, this child underwent cardiac catheterization at age7 years. A resting left ventricular outflow tract peak systolic gradient of 11 mm Hg did not changesignificantly following isoprotereno1infusion. Although no resting right ventricular outflow gradient wasdetected, following the infusion of isoproterenol a 13 mm Hg gradient was evoked. Cineangiograms suggestedsevere hypertrophy and trabeculation of both right and left ventricular chambers. He has remained asymptomatic. Recent examination at age 16 demonstrated a diffuse apical impulseand a grade II/VI systolic murmur at the lower left sternal border. His ECG (Fig. 2) demonstrated type B WPW conduction. A two-dimensionalechocardiogram(2DE) (Fig. 3) demonstratedsevere concentric biventricular hypertrophy without evidence of outflow tract obstruction. Case3. The younger child of case1 was also noted to have asymptomatic cardiomegaly and a grade II/VI systolic murmur when he underwent cardiac catheterization at age 6 years. No resting or inducible gradients acrossthe left ventricular outflow tract were present. Following isoproterenol infusion, a 15 mm Hg gradient acrossthe right ventricular outflow tract was induced. Cineangio-
From the Division of Cardiovascular Diseases, William Beaumont Hospital. Reprint requests: Andrew M. Hauser, M.D., Division of Cardiovascular Diseases, William Beaumont Hospital, 3601 W. Thirteen Mile Rd., Royal Oak, MI 48072.
174
Brief
Communications
Case2. D.F., a 29-year-old man with rheumatic mitral valve disease,underwent mitral valve replacement with a No. 33 Carpentier-Edwards porcine valve. The postoperative course was uneventful. An ME performed before discharge showed mild enlargement of the LV (enddiastolic diameter of 5.9 cm) and paradoxical motion of the IVS (Fig. 2, A). Four weeksfollowing the operation the patient wasreadmitted for severedyspnea.Blood pressure was 90/50 mm Hg, heart rate was 128/min, and respirations were 29/min. He was lethargic, confused, and had rales over the entire lung fields. There were no murmurs. The ECG revealed sinus tachycardia. A repeat ME showed: (1) LV diameters and 1% paradoxical motion unchanged from previous examination; (2) diastolic fluttering of the posterior porcine leaflet; and (3) early diastolic anterior hump of the stents (Fig. 2, R). LV cineangiographyrevealed 4+ mitral regurgitation. During surgery the mitral bioprosthesis was found detached about 50 degreesaround the ring. The mitral valve was replaced, and the tricuspid valve, becauseof significant regurgitation, wasrepaired with a Carpentier anulus.The patient died after surgery becauseof persistent low output state. The most common ME finding reported in mitral prosthesisdetachment is LV volume overload with early postoperative normalization of septal motion4 However, this, is an indirect and nonspecificsign, since it can occur with a leaking mitral or aortic prosthesis,4and with both paraprosthetic and intraprosthetic regurgitation.? Furthermore, it is not always present, as for instance when right ventricular diastolic overload coexists, as in our second patient with significant tricuspid regurgitation. Another sign reported with dehiscent porcine mitral valve has been diastolic fluttering of porcine leaflets.? This finding is alsononspecific; in fact, it has been reported in native aortic valve regurgitation and in patients with torn porcine mitral leaflets.’ The sign we found here is a new one. Normally the bioprosthetic stent moves anteriorly during systole and posteriorly at the beginning of diastole (Figs. 1, A and 2, A). Conversely, in our patients the initial diastolic posterior movement was substituted by an early diastolic anterior hump (Figs. 1, B and 2, B). Probably this finding is the equivalent of the early diastolic hump describedby others”.” in casesof paravalvular leaking mechanicalprostheses.For this reason,and sinceit has not been reported in patients with other kinds of porcine mitral valve dysfunction, the early diastolic hump highly suggestsa dehiscent mitral bioprosthesis. Furthermore, since prosthetic mitral paravalvular regurgitation may be present without murmurs making clinical recognition difficult, ME may be an important diagnostictool for detecting this complication. REFERENCES
I. Alam M, Madrazo and two-dimensional valve dysfunction. 2. Salati A, Narducci
AC, Magilligan DJ, Goldstein S: M-mode echocardiographic features of porcine Am J Cardiol 432502, 1979. C, Pino PG, Chidichimo G, Jacovella G:
American
Heart
1984 Journal
Patologia delle protesi mitraliche porcine. Rilievi ecocardiografici in cinque casi con rigurgito intra-para-protesico. G Ital Cardiol 10:605, 1980. Horowitz MS, Goodman DJ, Hancock EW, Popp RL: Noninvasive diagnosis of complications of the mitral bioprosthesis. J Thorac Cardiovasc Surg 71:450, 1976. Feigenbaum H: Echocardiography. 3rd ed. Philadelphia, 1981, Lea & Febiger, p 313. Yuste P, Aza V, Minguez I, Asin Cardiel E, Martinez Bordiu C: Echocardiographic evaluation of mitral valve prosthesis malfunction. In Kazner E, editor: Ultrasonics in medicine. Amsterdam, 1975, Excerpta Medica, p 239. Bernal-Ramirez JA, Phillips JH: Echocardiographic study of malfunction of the Bjork-Shiley prosthetic heart valve in the mitral position. Am .J Cardiol 40:449, 1977.
Transumbilical balloon atrial septostomy for transposition of the great arteries in infants under the age of 60 hours N. Roguin, P. Sujov, J. Montag, M. Zeltzer, and E. Riss. Haifa and Nahariya, Israel Balloon atria1 septostomy (BAS)’ has dramatically improved life expectancy for infants with complete transposition of the great arteries (TGA). It is the purpose of this report to analyze our results with the transumbilical BAS approach and consider the optimal age to use the umbilical vein technique in babies with TGA. Ten patients below the age of 60 hours with complete TGA underwent cardiac catheterization during the period from February 1,1979, to August 30,1981, at the Rambam Medical Center, in Haifa, Israel. In the caseof 3 of the 10 patients, it was impossibleto perform the BAS through the umbilical vein route, and a saphenofemoraljunction cutdown technique wasemployed. The agesof the infants at the time of the procedure ranged from 16 to 56 hours (mean31 hours), the weight being from 2.2 to 3.8 kg (mean 3.2 kg). All the babieswere examined without sedation or anesthesia.In the three patients mentioned above, local anesthesiahad to be given prior to cutdown, the agesof these babies at the time being 16, 48, and 56 hours. At first, a No. 5 angiographic catheter was introduced through the umbilical vein-ductus venosusto the heart. Pressurerecordings and blood samplesfor oxygen determinations were taken In casethe diagnosisof TGA was not clear enough, cineangiography was necessary-either in the right or left ventricle. After confirmation of the diagnosis, a No. 5 Miller single-lumen balloon atria1 septostomy catheter (Edwards Laboratories, Santa Ana, From the Departments of Cardiology and Neonatology, Center, and Faculty of Medicine, Technion-Institute the Department of Neonatology, Nahariya Government Reprint Center,
requests: N. Roguin. Haifa, Israel.
M.D.,
Dept.
of Cardiology,
Rambam Medical of Technology: and Hospital. Rambam
Medical
Volume
107
Number
I
California) was introduced through the umbilical veinductus venosuswithout difficulty in the left atrium. The balloon wasthen inflated with 3 to 3.5 cc of 20% solution of contrast medium, and was rapidly jerked back to the inferior vena cava. Immediately after withdrawal it was advanced to the right atrium and then deflated rapidly. During this procedure, one hand was pressedgently over the umbilical area of the baby to control the withdrawal of the balloon catheter. This procedure was repeated from five to eight times. The catheter was then changed for a No. 5 catheter, and pressuresin the left and right atrium weretaken. A blood samplefor oxygen determinations was made in superior vena cava and/or aorta. During the entire process,a careful monitoring of blood gasesand temperature of the baby were done. All infants were treated with systemic antibiotics (penicillin and kanamytin) for 4 days as prophylaxis against sepsis.We carried out a complete investigation with cineangiography into the right and left ventricles and with an aortogram at the age of 3 or 4 months’; at the age of 6 months surgical intervention was necessary.During the entire period the babies were examined in our outpatient clinic twice a month; in addition to all the clinical signs we carefully controlled the level of plasma hemoglobin, and iron supplements were given when necessary. Out of eight patients examined under the age of 36 hours (mean 26.2 hours), the BAS was successfully performed in seven of them (87.5?;) through the transumbilical route without resorting to anesthesiaor premeditation. No perforation of the heart or sepsisor bleedingwere observed,while only one baby presentedabdominal distension,which persisted for 24 hours. After BAS there wasan increaseof the aortic or superior vena cava oxygen saturation of 17% mean, as well as an equalization of atria1 pressures.The babies all showedimproved clinical and metabolical condition, and were dischargedfrom the hospital 5 days after the procedure. Out of seven babies who underwent transumbilical BAS, five had simple TGA: one had TGA with pulmonic stenosisand one had TGA with large ventricular septal defect. One baby (TGA and pulmonic stenosis)died at the ageof 40 days from aspiration pneumonia.Six babieswere reinvestigated at the age of 4 months and underwent surgical correction at the age 6 months: four had a successfulbaffle procedure and two died-one with a simple TGA after an “anatomic” procedure, and the secondafter a baffle procedure and closureof the ventricular septal defect. In five out of the six babies,the atria1 septal defect was measured by the surgeon and ranged from 1.1to 14 mm in diameter, while in the patient who died at the ageof 40 days, the diameter of the atria1 septal defect was 12 mm. The ductus venosus constitutes a direct connection betweenthe umbilical vein and the inferior vena cava. It is about half the size of the umbilical vein,3 functioning asa low resistancebypassto the heart, and is unresponsiveto changesin the blood gasesor acid-baseequilibrium. It is, however, constricted by high concentration of epineph-
Brief
Communications
175
rine.4The postnatal closureof the ductus venosusappears to be a result of decreasein pressurein the portal sinus following cessation of flow in the umbilical vein and resulting in retraction and narrowing of its origin while assuminga slitlike appearance.The functional closure of the ductus venosusoccurs during the first minutes after birth and is not complete. The organic closure of the ductus venosusbeginsat its narrow origin, with proliferation of the obliteration tissue in specially structured border strips. The closure is completed between the fifteenth and twentieth day.3 Owing to the possibility of liver and portal vein infection, Rudolph4is not in favor of performing umbilical vein cardiac catheterization after the third day of life. He suggestsmaking a brief attempt to manipulate the catheter through the ductus venosus,and if this doesnot prove satisfactory, he prefers to dissectthe groin. We tend to sharehis view that there is little hope of successin the transumbilical approach, and therefore will not consider it after the third day of life. At the present time there are three technical possibilities of performing balloon septostomyin babieswith TGA: the femoral vein percutaneous technique, the saphenofemoral junction cutdown technique, and the transumbilical approach (in addition, there is also the possibility of using an axillary vein cutdown technique, making it more difficult, however, to perform BAS through this route). The percutaneoustechnique requires a No. 6 or 7 sheath for the femoral vein to introduce a balloon septostomy catheter. In capablehandsit is a goodtechnique, but often could unfortunately be traumatic for the baby, producing an inguinal hematoma. Should the femoral vein be posterior in relation to the femoral artery, it may be difficult or even impossibleto adopt this approach. Keane et a1.5reported an incidence of 16% of iliac vein or inferior vena cava thrombosiswhen this technique was used, and this complication was even two or three times higher in infants who underwent BAS than in those who did not. However, this was not of statistical significance owing to the relatively small number of patients involved. One factor suspectedof predisposingto inferior vena cava thrombosis is prolonged use of indwelling venous catheters; however, this factor was present in only 1 of the 12 patients reported.5Mathews et a1.6reported that the useof balloon catheters-either septostomy or flow-directedmay have contributed to venous thrombosis diagnosedin 19 (80%) of 22 patients. Venous thrombosis is a serious complication in patients with TGA requiring repetitive cardiac catheterization, and both the femoral veins will no longer be available. In addition to the technical points mentioned, other severecomplications have beenreported following inferior vena cava thrombosis, including pulmonary emboli and thrombosis and renal and adrenal veins. Technical skill is essentialin performing the saphenofemoral junction cutdown, as otherwise bleeding may result and repair of the vein after septostomy could produce someedemaand/or cyanosisof the leg. Skin infections are not uncommon, and sepsishas been noted,7 as well as thrombosis of the femoral vein or inferior vena cava.
176
Brief
Communications
However, the incidence of late complications is relatively low (l.2%).5 Porter et a1.7reported an experience with cardiac catheterization in the first week of life, and concluded that the umbilical vein route took 30 minutes lessthan either the percutaneousor cutdown techniques. We also feel that time is a factor of great importance in very ill babieswith TGA. To the best of our knowledge, the first transumbilical BAS in a baby 56 hours of agewas reported by Abinader et a1.8from our institution. The secondcase was reported by Romney et a1.3Newfeld et al.“’ reported 16 casesof transumbilical BAS in babies belowthe ageof 4 days; in 20% of casesit wasnot possible to use the umbilical approach. However, the authors did not correlate the age of the babies with failure of this method. No infection, bleeding, or liver damage was reported. We have undergonethe experienceof performing transumbilical BAS with successin 7 out of 10babiesbelow the ageof 60 hours, and in sevenout of eight cases(87.5%) in babiesbelow the ageof 36 hoursthe procedure wascarried out quickly and without undue complications. No anesthesia or premeditation was used. In treating babieswith suspectedTGA, we do not recommend introducing any umbilical vein or artery catheter before the procedure in order to avoid manipulation and possibilities of previous hemorrageand infection in the umbilical area. In conclusion,we recommendtransumbilical BAS as the method of choicefor babieswith TGA, especiallythose under the age of 36 hourswhen the rate of successis approximately 90%. The procedure is safe, and can be performed easily and quickly, leaving both saphenousveins available for future recatheterization. This study stressesthe need for early treatment of patients suspectedof having TGA.
REFERENCES 1. Rashkind 2.
3. 4. 5.
6.
7.
8. 9.
19.
WJ, Miller WW: Creation of an atria1 septal defect without thoractomy. JAMA 196:991, 1966. Leenage R, Aquetti A, Graham G, Taylor J, Macartney FJ: Factors influencing survival after balloon atria1 septostomy for complete transposition of great arteries. Br Heart J 15:559, 1981. Lind J: Human fetal and neonatal circulation. Some structural and functional aspects. Eur J. Cardiol 5(3):265, 1977. Rudoloh AM: Conaenital diseases of the heart. Chicago,. 1974, Year Book Medical Publishers, Inc, p 18. Keane JF, Lang P, Newburger J, Fyler DC: Iliac vein-inferior caval thrombosis after cardiac catheterization in infancy. Pediatr Cardiol 1:257, 1980. Mathews RA, Park SC, Neches WH, Fricker FJ, Lenox CC, Zuberbuhler JR: Iliac venous thrombosis in infants and children after cardiac catheterization. Cathet Cardiovasc 5:67, 1979. Porter CJ, Gillete PC, Mullins CE, McNamara DC: Cardiac catheterization in the neonate. A comparison of three techniques. J Pediatr 93(1):97, 1978. Abinader E, Zeltzer M, Riss E: Transumbilical atria1 septostomy in the newborn. Am J Dis Child 119:354, 1970. Romney D, Katzuni E, Aygen MM: Transumbilical balloon atria1 septostomy in a case of transposition of the great vessels with situs inversus totalis. Isr J Med Sci 8:529, 1972. Newfeld EA, Purcell C, Paul MH, Cole RV, Muster AJ: Transumbilical balloon atria1 septostomy in 16 infants with transposition of the great arteries. Pediatrics 54:495, 1974.
American
Familial hypertrophic preexcitation
cardiomyopathy
January, 1994 Heart Journal
and
Andrew M. Hauser, M.D., Seymour Gordon, M.D., and Gerald C. Timmis, M.D. Royal Oak, Mich. The familial occurrence of hypertrophic cardiomyopathy (HCM) has been noted in numerous reviews,‘,2 and an autosomal dominant mode of inheritance with variable expressivity and penetrance has been proposed.‘,” The familial occurrence of Wolff-Parkinson-White (WPW) conduction is rare,4 an autosomal dominant mode of inheritance alsohaving been suggested.“,”Although HCM is at times associated with WPW conduction,2,“-7the coexistence of HCM and WPW conduction in multiple family membersof the samegeneration hasbeen reported only twice.“,” We now report a third family in which two generations manifested coexisting WPW conduction and HCM of severe and diffuse nature. Case 1. The mother of the two afflicted children described below presented at age 26 years with a 3-year history of progressive dyspnea and cardiomegaly. Her ECG (Fig. 1) demonstrated morphologic features of type B WPW conduction. Progressive heart failure led to her death 1 year after presentation. Available autopsy records reported severe diffuse cardiac hypertrophy and normal coronary arteries. The patient bore two sons;cases2 and 3 below. Case 2. Becauseof an asymptomatic systolic murmur and cardiomegaly on chest x-ray examination, this child underwent cardiac catheterization at age7 years. A resting left ventricular outflow tract peak systolic gradient of 11 mm Hg did not changesignificantly following isoprotereno1infusion. Although no resting right ventricular outflow gradient wasdetected, following the infusion of isoproterenol a 13 mm Hg gradient was evoked. Cineangiograms suggestedsevere hypertrophy and trabeculation of both right and left ventricular chambers. He has remained asymptomatic. Recent examination at age 16 demonstrated a diffuse apical impulseand a grade II/VI systolic murmur at the lower left sternal border. His ECG (Fig. 2) demonstrated type B WPW conduction. A two-dimensionalechocardiogram(2DE) (Fig. 3) demonstratedsevere concentric biventricular hypertrophy without evidence of outflow tract obstruction. Case3. The younger child of case1 was also noted to have asymptomatic cardiomegaly and a grade II/VI systolic murmur when he underwent cardiac catheterization at age 6 years. No resting or inducible gradients acrossthe left ventricular outflow tract were present. Following isoproterenol infusion, a 15 mm Hg gradient acrossthe right ventricular outflow tract was induced. Cineangio-
From the Division of Cardiovascular Diseases, William Beaumont Hospital. Reprint requests: Andrew M. Hauser, M.D., Division of Cardiovascular Diseases, William Beaumont Hospital, 3601 W. Thirteen Mile Rd., Royal Oak, MI 48072.