- Email: [email protected]
Massive calcification of a porcine bioprosthesis in the aortic valve position and the role of calcium supplements
Volume 121 Number 6, Part 1
can be introduced into an 8F sheath. A 2 mm string loop is attached to the center of the occluder; the loop is closed by a 1 mm knot (“button”). The counter-occluder is made up of a rhomboid-shaped polyurethane foam mounted on a Teflon-coated wire skeleton. A rubber piece is sutured in its center and becomes a buttonhole. The delivery system consists of: (1) a loading wire, which is a Teflon-coated 0.028-inch guide wire (Cook Inc., Bloomington, Ind.); (2) a folded 0.005-inch nylon thread, the loop of which passes through the loop in the center of the occluder; (3) an 8F long sheath (Cordis Corp., Miami Lakes, Fla.); and (4) a 7F end-hole catheter (pusher) that is used to advance the oceluder and counter-occluder within the sheath. The infant was premeditated with a mixture of meperidine, and chlorpromazine, and no general anesthesia was used. Percutaneous right heart catheterization data were obtained in the usual manner and the data were similar to those obtained at the initial catheterization. Main pulmonary artery and left atria1 cineangiograms were performed that reconfirmed the presence of an ASD. The right heart catheter was exchanged with 5F Edwards ballooon septostomy catheter (Baxter Healthcare Corp., Edwards Division, Santa Ana, Calif.) and the stretched diameter of the ASD was measured by passing progressively larger balloons in a manner described by King et al.* The defect measured 10 mm; it measured 7 mm on an echocardiographic study (Fig. 1, A). A 25 mm device was chosen for transcatheter closure. At this juncture, 360 units of heparin was administered, and a femoral arterial line to monitor the systemic pressure during the procedure was placed. The 6F sheath in the femoral vein was exchanged over a guide wire with a long 8F Cordis sheath with the help of an 8F catheter, and the tip of the sheath was positioned in the mid-left atrium. The occluder was delivered into the left atrium via the 8F sheath already in place. The counter-occluder was delivered into the right atrium. The occluder and the counteroccluder were buttoned across the ASD (Table I). The delivery system was disconnected from the device. A main pulmonary artery cineangiogram was performed 1.5 minutes after device implantation. No residual left-to-right shunt across the atria1 septum was detected on the levophase of the cineangiogram. Echo-Doppler studies to visualize the atria1 septal defect (Fig. 1, A) and to demonstrate flow across it were performed prior to and immediately after (Fig. 1, B) device implantation and were repeated 0.5,3, and 6 months following the procedure. The device was intact (Fig. 1, C and D) and showed no evidence for left-to-right shunt across the atria1 septum, either by pulsed or color Doppler evaluation at the time of follow-up studies. The device was also well visualized on a plain chest roentgenogram (Fig. 2). The infant improved clinically and the anticongestive measures were discontinued 1 month after device implantation. Transcatheter ASD closure devices used by King,a Rashkind, and Lock3z4 require 23, 16, and 1lF sheaths, respectively, for delivery. Because of the requirement of large size catheter delivery systems, these devices have not been used in small infants. The smallest child in whom ASD device implantation was tried weighed 11.0 kg,7 and in this infant the procedure was not successful. Because of the small size of the catheter delivery system (8F) required
Brief Communications
1829
to deliver the ASD device and because of a simple mechanism for implantation of the device across the ASD, we have chosen to use this device to close the ASD in this small-sized infant, with success. The device remained in position during the 6-month follow-up period and no residual shunt was documented by pulsed or color Doppler studies. We conclude that implantation of the Sideris’ “buttoned” double-disk device to close the ASD in small infants is feasible, effective, and safe. Further clinical trials are indicated. REFERENCES
1. King TD, Thompson SL, Steiner C, Mills NL. Secundum atria1 septal defect: nonoperative closure. J Am Med Assoc 1976;235:2506-9. 2. Rashkind WJ. Transcatheter treatment of congenital heart disease. Circulation 1983;67:711-6. 3. Lock JE, Rome JJ, Davis R, Van Praagh S, Perry SB, Van Praagh R, Keane JF. Transcatheter closure of atria1 sental defects: experimental studies. Circulation 1989;79:1091-9. 4. Lock JE. Hellenbrand WE. Latson L. Mullins CE. Benson L. Rome JJ. Clamshell umbrella closure of atria1 se&l defects: initial experience [Abstract]. Circulation 1989;80:11-592. 5. Sideris EB, Sideris SE, Foulkes JP, Ehly RL, Smith JE, Guide RE. Transvenous atria1 septal occlusion in piglets using a “buttoned” double disk device. Circulation 1990;81:312-8. 6. Sideris EB, Sideris SE, Thonopoulos BD, Ehly RL, Foulkes JP. Transvenous atria1 septal defect occlusion by the buttoned device. Am J Cardiol 1990;66:1524-6. 7. Hellenbrand WE, Fahey JT, McGowan FY, Weltin GG, Kleinman CS. Transesophageal echocardiographic guidance of transcatheter closure of atria1 septal defect.Am J Cardiol 1990;66:207-13. 8. King TD, Thompson SL, Mills NL. Measurements of atria1 septal defect during cardiac catheterization: experimental and clinical results. Am J Cardiol 1978;41:537-42.
Massive calcification of a porcine bioprosthesis in the aortic valve position and the role of calcium supplements Heinrich G. Klues, MD, Lowell S. Statler, MD, Robert B. Wallace, MD, and William C. Roberts, Washington, D.C., and Bethesda, Md.
MD.
The development of calcific deposits in porcine aortic valve cusps used in bioprostheses for cardiac valve replacement in humans is well recognized. Often the calcific deposits cause tearing of the porcine aortic valve cusps and the tearing may lead to severe valvular regurgitation. The calcific deposits on occasion may be large enough to decrease the mobility of the cusps and the immobility may lead to varying degrees of dysfunction. Bioprosthetic regurgitation, however, is a more common major complication of From the Division
of Cardiology, Department of Medicine, and the Department of Surgery, Georgetown University Medical Center; and the Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health. Reprint BI-NIH,
4/4/28762
requests: William C. Roberts, Bethesda, MD 20892.
MD,
Bldg.
10, Room
2N258,
NHL-
June
1830
Brief Communications
American
Heart
1991 Journal
Fig. 1. Continuous-wave Doppler tracing obtained from the left ventricular apex demonstrating a 6 m/ set jet across the aortic valve, corresponding to a calculated peak systolic gradient of approximately 140 mm Hg between the left ventricle and aorta. The tracing also shows a mild aortic regurgitation jet.
Fig. 2. Massively calcified bioprosthetic valve removed from the aortic valve position 126 months after implantation. Left panel, Radiograph; center panel, view from the aortic aspect; right panel, view from the ventricular aspect. It is extremely unusual to have calcific deposits on both ventricular and aortic aspects of the cusps; they are usually only found on the aortic aspects.
bioprosthetic replacement than is stenosis. In this report we describe massive calcific deposits with resulting extreme stenosis unassociated with significant regurgitation in a porcine bioprosthesis in the aortic valve position, and we discuss the possible relation of daily calcium supplements on the amount of bioprosthetic calcific deposits. A 74-year-old woman had aortic valve replacement for a stenotic (47 mm Hg peak systolic pressure gradient between the left ventricle and the ascending aorta) congenitally bicuspid aortic valve stenosis in September 1978 when she was 64 years old. The native valve was replaced with a porcine bioprosthesis (Hancock 27 mm, Johnson & Johnson Cardiovascular, King of Prussia, Pa.). She was asymptomatic thereafter until January 1989, when while she was on a cruise, she noted dyspnea upon walking up stairs on the ship. Three days later, she was in acute pulmonary edema and was hospitalized. An echocardiogram (Fig. 1) disclosed a peak systolic pressure gradient between
the left ventricle and the aorta of approximately 140 mm Hg. At cardiac catheterization, the bioprosthesis in the aort,ic valve position could not be crossed by the catheter despite repeated attempts. Five days after the onset of the dyspnea on the ship, the Hancock bioprosthesis in the aortic valve position was replaced with a Carpentier-Edwards bioprosthesis (29 mm, Baxter Healthcare Corp., Edwards Division, Santa Ana, Calif.). Eighteen months later (August 1990) she was asymptomatic and was working daily in her garden. The operatively excised Hancock bioprosthesis is shown in Fig. 2. Each cusp is heavily calcified, virtually immobile, and the deposits are present on both aortic and ventricular aspects of the cusps. No cusp is torn or perforated. Early postoperatively it was learned that this woman had been taking a calcium supplement daily for no specific medical indication for at least 20 years, including each of the 3769 days that the porcine bioprosthesis had been in the aortic valve position. Each Caltrate tablet (Le-
Volume 121 Number 6, Part
Brief
1
derle Laboratories, Pearl River, N.Y.) she took contained 1500 mg of calcium carbonate. Thus she took at least 5654 gm of Caltrate during the 3769 days. Each Caltrate tablet contains 600 mg of elemental calcium; therefore she took 2261 gm of elemental calcium during this 126-month postoperative period. Just before aortic valve replacement the blood urea nitrogen was 18 mg/dl and the creatinine was 1.0 mg/dl. The unusual features of the patient described herein are the massive quantity of calcific deposits on the cusps of the porcine bioprosthesis, the degree of the pressure gradient across the bioprosthesis, the lack of tearing or perforations in the bioprosthetic cusps despite the huge calcific deposits, and the possibility that the taking of daily calcium supplements played a role in the development of the heavy calcific deposits. Search of reports describing late follow-up of patients having bioprostheses in the aortic valve position did not uncover any reported patients having a peak systolic pressure gradient >lOO mm Hg across a bioprosthesis in the aortic valve position. Furthermore, we have not encountered any bioprostheses in any valve position previously having as extensive calcific deposits as were present in the bioprosthesis of the patient described herein. At least one other report has attempted to link heavy calcific deposits in a bioprosthesis to the taking of calcium supplements. Moront and Katz1 described a 72-year-old woman who developed massive calcific deposits on a bioprosthesis in the mitral valve position in an l&month period and the mean diastolic pressure gradient across the bioprosthesis was 19 mm Hg. This woman also took calcium carbonate supplements daily. It would appear from the study of the patient by Moront and Katz and from the study of the patient described herein that persons having bioprostheses should not take calcium supplements. REFERENCE
1.
Moront MG, Katz NM. Early degeneration of a porcine aortic valve bioprosthesis in the mitral valve position in an elderly woman and its association with long-term calcium carbonate therapy. Am J Cardiol 1987;59:1006-7.
Total anomalous pulmonary venous return: Diagnosis by transesophageal echocardiography Angel Jesus Oliver Manuel Javier
Romero-Cardenas, MD, Vargas-Barron, MD, Maria Rylaarsdam, MD, Stumper, MD, Manuel Villegas, MD, A. Bandin, MD, Candace Keirns, MD, and Molina, MD. Mexico City, Mexico
From the Department logia Ignacio Chivez. Reprint Institute Tlalpan 4/4/28837
of Echocardiography,
Instituto
requests: Jestis Vargas-Barrbn, MD, Dept. National de Cardioloria, Imacio Chlvez. 14080, M&&m, D.F., Mexico.
National
de Cardjo-
of Echocardiography, Juan Bad&o No. I.
Communications
183
1
Total anomalous pulmonary venous return (TAPVR) is defined as the congenital anomaly in which the pulmonary veins are not connected to the left atrium. A classification of the level at which the anomalous connection occurs has been proposed as: (1) supracardiac if it is through the right superior vena cava, arch of the azygos vein, innominate vein, or left superior vena cava; (2) cardiac if it is directly into the coronary sinus or the right atrium; or (3) infracardisc if drainage is into the portal vein, hepatic veins, or inferior vena cava. Mixed anomalous connections exist that include one or more of the three types described.l TAPVR has been found in up to 2 % of 800 autopsies of congenital cardiopathies, with connection to the coronary sinus representing 16% of the cases and connection to the right atrium representing 15 % .l, 2 This condition produces volume overload of the right cavities and pulmonary hypertension that can be detected by two dimensional (2-D) echocardiography. Moreover, this technique permits identification of partial or complete confluence of the pulmonary veins in a venous collector or supernumerary chamber behind the left atrium.3 Doppler echocardiography has made it possible to observe abnormal characteristics of flow produced by anomalous pulmonary venous return as well as patterns of turbulence created by obstruction of flo~,~ and color-coded Doppler echocardiography has facilitated location of these abnormal flows. Transesophageal echocardiography (TEE) brings us in close proximity to the heart without the technical drawbacks of transthoracic exploration. At the same time it facilitates examination of posterior structures including the left atrium and appendage and the return and flow of the pulmonary veins.5, 6 The purpose of this report is to describe the findings obtained by TEE in three patients with TAPVR. Case No. 1. A 16-year-old girl weighing 42 kg had a history of cyanosis and heart murmur from the age of 2 years. Later she developed exertional dyspnea and fatigue. Physical examination on admission revealed clubbing of the fingers and cyanosis (grade +++). No murmurs were heard over the heart; a protosystolic snap and loud split second heart sound were detected in the pulmonary area. The chest x-ray film showed grade II cardiomegaly owing to enlargement of the right cavities, bulging of the pulmonary artery, and increased pulmonary vasculature. In the electrocardiogram (ECG), enlargement of the right cavities was evident. Transthoracic echocardiography showed dilatation of the right cavities and the presence of a venous collector behind the left atrium. Anomalous pulmonary venous return to the right atrium was suspected. Cardiac catheterization was performed with injection of contrast medium into the main pulmonary artery. A retrocardiac venous collector was observed; it was not possible to identify the right superior pulmonary vein. Pressures registered in the pulmonary artery were systolic 56 mm Hg, diastolic 26 mm Hg, and mean 39 mm Hg. Before surgery TEE was performed with the patient under general anesthesia using a 5 MHz transducer and Sonoline CF echocardiograph (Siemens AG, Munich, Germany). A structure was identified in front of the esophagus and behind the left atrium (venous collector) into which the left pulmonary veins drained. Right pulmonary veins drained into the right atrium-the superior chamber close to the superior vena
can be introduced into an 8F sheath. A 2 mm string loop is attached to the center of the occluder; the loop is closed by a 1 mm knot (“button”). The counter-occluder is made up of a rhomboid-shaped polyurethane foam mounted on a Teflon-coated wire skeleton. A rubber piece is sutured in its center and becomes a buttonhole. The delivery system consists of: (1) a loading wire, which is a Teflon-coated 0.028-inch guide wire (Cook Inc., Bloomington, Ind.); (2) a folded 0.005-inch nylon thread, the loop of which passes through the loop in the center of the occluder; (3) an 8F long sheath (Cordis Corp., Miami Lakes, Fla.); and (4) a 7F end-hole catheter (pusher) that is used to advance the oceluder and counter-occluder within the sheath. The infant was premeditated with a mixture of meperidine, and chlorpromazine, and no general anesthesia was used. Percutaneous right heart catheterization data were obtained in the usual manner and the data were similar to those obtained at the initial catheterization. Main pulmonary artery and left atria1 cineangiograms were performed that reconfirmed the presence of an ASD. The right heart catheter was exchanged with 5F Edwards ballooon septostomy catheter (Baxter Healthcare Corp., Edwards Division, Santa Ana, Calif.) and the stretched diameter of the ASD was measured by passing progressively larger balloons in a manner described by King et al.* The defect measured 10 mm; it measured 7 mm on an echocardiographic study (Fig. 1, A). A 25 mm device was chosen for transcatheter closure. At this juncture, 360 units of heparin was administered, and a femoral arterial line to monitor the systemic pressure during the procedure was placed. The 6F sheath in the femoral vein was exchanged over a guide wire with a long 8F Cordis sheath with the help of an 8F catheter, and the tip of the sheath was positioned in the mid-left atrium. The occluder was delivered into the left atrium via the 8F sheath already in place. The counter-occluder was delivered into the right atrium. The occluder and the counteroccluder were buttoned across the ASD (Table I). The delivery system was disconnected from the device. A main pulmonary artery cineangiogram was performed 1.5 minutes after device implantation. No residual left-to-right shunt across the atria1 septum was detected on the levophase of the cineangiogram. Echo-Doppler studies to visualize the atria1 septal defect (Fig. 1, A) and to demonstrate flow across it were performed prior to and immediately after (Fig. 1, B) device implantation and were repeated 0.5,3, and 6 months following the procedure. The device was intact (Fig. 1, C and D) and showed no evidence for left-to-right shunt across the atria1 septum, either by pulsed or color Doppler evaluation at the time of follow-up studies. The device was also well visualized on a plain chest roentgenogram (Fig. 2). The infant improved clinically and the anticongestive measures were discontinued 1 month after device implantation. Transcatheter ASD closure devices used by King,a Rashkind, and Lock3z4 require 23, 16, and 1lF sheaths, respectively, for delivery. Because of the requirement of large size catheter delivery systems, these devices have not been used in small infants. The smallest child in whom ASD device implantation was tried weighed 11.0 kg,7 and in this infant the procedure was not successful. Because of the small size of the catheter delivery system (8F) required
Brief Communications
1829
to deliver the ASD device and because of a simple mechanism for implantation of the device across the ASD, we have chosen to use this device to close the ASD in this small-sized infant, with success. The device remained in position during the 6-month follow-up period and no residual shunt was documented by pulsed or color Doppler studies. We conclude that implantation of the Sideris’ “buttoned” double-disk device to close the ASD in small infants is feasible, effective, and safe. Further clinical trials are indicated. REFERENCES
1. King TD, Thompson SL, Steiner C, Mills NL. Secundum atria1 septal defect: nonoperative closure. J Am Med Assoc 1976;235:2506-9. 2. Rashkind WJ. Transcatheter treatment of congenital heart disease. Circulation 1983;67:711-6. 3. Lock JE, Rome JJ, Davis R, Van Praagh S, Perry SB, Van Praagh R, Keane JF. Transcatheter closure of atria1 sental defects: experimental studies. Circulation 1989;79:1091-9. 4. Lock JE. Hellenbrand WE. Latson L. Mullins CE. Benson L. Rome JJ. Clamshell umbrella closure of atria1 se&l defects: initial experience [Abstract]. Circulation 1989;80:11-592. 5. Sideris EB, Sideris SE, Foulkes JP, Ehly RL, Smith JE, Guide RE. Transvenous atria1 septal occlusion in piglets using a “buttoned” double disk device. Circulation 1990;81:312-8. 6. Sideris EB, Sideris SE, Thonopoulos BD, Ehly RL, Foulkes JP. Transvenous atria1 septal defect occlusion by the buttoned device. Am J Cardiol 1990;66:1524-6. 7. Hellenbrand WE, Fahey JT, McGowan FY, Weltin GG, Kleinman CS. Transesophageal echocardiographic guidance of transcatheter closure of atria1 septal defect.Am J Cardiol 1990;66:207-13. 8. King TD, Thompson SL, Mills NL. Measurements of atria1 septal defect during cardiac catheterization: experimental and clinical results. Am J Cardiol 1978;41:537-42.
Massive calcification of a porcine bioprosthesis in the aortic valve position and the role of calcium supplements Heinrich G. Klues, MD, Lowell S. Statler, MD, Robert B. Wallace, MD, and William C. Roberts, Washington, D.C., and Bethesda, Md.
MD.
The development of calcific deposits in porcine aortic valve cusps used in bioprostheses for cardiac valve replacement in humans is well recognized. Often the calcific deposits cause tearing of the porcine aortic valve cusps and the tearing may lead to severe valvular regurgitation. The calcific deposits on occasion may be large enough to decrease the mobility of the cusps and the immobility may lead to varying degrees of dysfunction. Bioprosthetic regurgitation, however, is a more common major complication of From the Division
of Cardiology, Department of Medicine, and the Department of Surgery, Georgetown University Medical Center; and the Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health. Reprint BI-NIH,
4/4/28762
requests: William C. Roberts, Bethesda, MD 20892.
MD,
Bldg.
10, Room
2N258,
NHL-
June
1830
Brief Communications
American
Heart
1991 Journal
Fig. 1. Continuous-wave Doppler tracing obtained from the left ventricular apex demonstrating a 6 m/ set jet across the aortic valve, corresponding to a calculated peak systolic gradient of approximately 140 mm Hg between the left ventricle and aorta. The tracing also shows a mild aortic regurgitation jet.
Fig. 2. Massively calcified bioprosthetic valve removed from the aortic valve position 126 months after implantation. Left panel, Radiograph; center panel, view from the aortic aspect; right panel, view from the ventricular aspect. It is extremely unusual to have calcific deposits on both ventricular and aortic aspects of the cusps; they are usually only found on the aortic aspects.
bioprosthetic replacement than is stenosis. In this report we describe massive calcific deposits with resulting extreme stenosis unassociated with significant regurgitation in a porcine bioprosthesis in the aortic valve position, and we discuss the possible relation of daily calcium supplements on the amount of bioprosthetic calcific deposits. A 74-year-old woman had aortic valve replacement for a stenotic (47 mm Hg peak systolic pressure gradient between the left ventricle and the ascending aorta) congenitally bicuspid aortic valve stenosis in September 1978 when she was 64 years old. The native valve was replaced with a porcine bioprosthesis (Hancock 27 mm, Johnson & Johnson Cardiovascular, King of Prussia, Pa.). She was asymptomatic thereafter until January 1989, when while she was on a cruise, she noted dyspnea upon walking up stairs on the ship. Three days later, she was in acute pulmonary edema and was hospitalized. An echocardiogram (Fig. 1) disclosed a peak systolic pressure gradient between
the left ventricle and the aorta of approximately 140 mm Hg. At cardiac catheterization, the bioprosthesis in the aort,ic valve position could not be crossed by the catheter despite repeated attempts. Five days after the onset of the dyspnea on the ship, the Hancock bioprosthesis in the aortic valve position was replaced with a Carpentier-Edwards bioprosthesis (29 mm, Baxter Healthcare Corp., Edwards Division, Santa Ana, Calif.). Eighteen months later (August 1990) she was asymptomatic and was working daily in her garden. The operatively excised Hancock bioprosthesis is shown in Fig. 2. Each cusp is heavily calcified, virtually immobile, and the deposits are present on both aortic and ventricular aspects of the cusps. No cusp is torn or perforated. Early postoperatively it was learned that this woman had been taking a calcium supplement daily for no specific medical indication for at least 20 years, including each of the 3769 days that the porcine bioprosthesis had been in the aortic valve position. Each Caltrate tablet (Le-
Volume 121 Number 6, Part
Brief
1
derle Laboratories, Pearl River, N.Y.) she took contained 1500 mg of calcium carbonate. Thus she took at least 5654 gm of Caltrate during the 3769 days. Each Caltrate tablet contains 600 mg of elemental calcium; therefore she took 2261 gm of elemental calcium during this 126-month postoperative period. Just before aortic valve replacement the blood urea nitrogen was 18 mg/dl and the creatinine was 1.0 mg/dl. The unusual features of the patient described herein are the massive quantity of calcific deposits on the cusps of the porcine bioprosthesis, the degree of the pressure gradient across the bioprosthesis, the lack of tearing or perforations in the bioprosthetic cusps despite the huge calcific deposits, and the possibility that the taking of daily calcium supplements played a role in the development of the heavy calcific deposits. Search of reports describing late follow-up of patients having bioprostheses in the aortic valve position did not uncover any reported patients having a peak systolic pressure gradient >lOO mm Hg across a bioprosthesis in the aortic valve position. Furthermore, we have not encountered any bioprostheses in any valve position previously having as extensive calcific deposits as were present in the bioprosthesis of the patient described herein. At least one other report has attempted to link heavy calcific deposits in a bioprosthesis to the taking of calcium supplements. Moront and Katz1 described a 72-year-old woman who developed massive calcific deposits on a bioprosthesis in the mitral valve position in an l&month period and the mean diastolic pressure gradient across the bioprosthesis was 19 mm Hg. This woman also took calcium carbonate supplements daily. It would appear from the study of the patient by Moront and Katz and from the study of the patient described herein that persons having bioprostheses should not take calcium supplements. REFERENCE
1.
Moront MG, Katz NM. Early degeneration of a porcine aortic valve bioprosthesis in the mitral valve position in an elderly woman and its association with long-term calcium carbonate therapy. Am J Cardiol 1987;59:1006-7.
Total anomalous pulmonary venous return: Diagnosis by transesophageal echocardiography Angel Jesus Oliver Manuel Javier
Romero-Cardenas, MD, Vargas-Barron, MD, Maria Rylaarsdam, MD, Stumper, MD, Manuel Villegas, MD, A. Bandin, MD, Candace Keirns, MD, and Molina, MD. Mexico City, Mexico
From the Department logia Ignacio Chivez. Reprint Institute Tlalpan 4/4/28837
of Echocardiography,
Instituto
requests: Jestis Vargas-Barrbn, MD, Dept. National de Cardioloria, Imacio Chlvez. 14080, M&&m, D.F., Mexico.
National
de Cardjo-
of Echocardiography, Juan Bad&o No. I.
Communications
183
1
Total anomalous pulmonary venous return (TAPVR) is defined as the congenital anomaly in which the pulmonary veins are not connected to the left atrium. A classification of the level at which the anomalous connection occurs has been proposed as: (1) supracardiac if it is through the right superior vena cava, arch of the azygos vein, innominate vein, or left superior vena cava; (2) cardiac if it is directly into the coronary sinus or the right atrium; or (3) infracardisc if drainage is into the portal vein, hepatic veins, or inferior vena cava. Mixed anomalous connections exist that include one or more of the three types described.l TAPVR has been found in up to 2 % of 800 autopsies of congenital cardiopathies, with connection to the coronary sinus representing 16% of the cases and connection to the right atrium representing 15 % .l, 2 This condition produces volume overload of the right cavities and pulmonary hypertension that can be detected by two dimensional (2-D) echocardiography. Moreover, this technique permits identification of partial or complete confluence of the pulmonary veins in a venous collector or supernumerary chamber behind the left atrium.3 Doppler echocardiography has made it possible to observe abnormal characteristics of flow produced by anomalous pulmonary venous return as well as patterns of turbulence created by obstruction of flo~,~ and color-coded Doppler echocardiography has facilitated location of these abnormal flows. Transesophageal echocardiography (TEE) brings us in close proximity to the heart without the technical drawbacks of transthoracic exploration. At the same time it facilitates examination of posterior structures including the left atrium and appendage and the return and flow of the pulmonary veins.5, 6 The purpose of this report is to describe the findings obtained by TEE in three patients with TAPVR. Case No. 1. A 16-year-old girl weighing 42 kg had a history of cyanosis and heart murmur from the age of 2 years. Later she developed exertional dyspnea and fatigue. Physical examination on admission revealed clubbing of the fingers and cyanosis (grade +++). No murmurs were heard over the heart; a protosystolic snap and loud split second heart sound were detected in the pulmonary area. The chest x-ray film showed grade II cardiomegaly owing to enlargement of the right cavities, bulging of the pulmonary artery, and increased pulmonary vasculature. In the electrocardiogram (ECG), enlargement of the right cavities was evident. Transthoracic echocardiography showed dilatation of the right cavities and the presence of a venous collector behind the left atrium. Anomalous pulmonary venous return to the right atrium was suspected. Cardiac catheterization was performed with injection of contrast medium into the main pulmonary artery. A retrocardiac venous collector was observed; it was not possible to identify the right superior pulmonary vein. Pressures registered in the pulmonary artery were systolic 56 mm Hg, diastolic 26 mm Hg, and mean 39 mm Hg. Before surgery TEE was performed with the patient under general anesthesia using a 5 MHz transducer and Sonoline CF echocardiograph (Siemens AG, Munich, Germany). A structure was identified in front of the esophagus and behind the left atrium (venous collector) into which the left pulmonary veins drained. Right pulmonary veins drained into the right atrium-the superior chamber close to the superior vena