Volume
124
Number
3
Brief Communications
Delineation of obstruction in total anomalous pulmonary venous connection utilizing magnetic resonance imaging Jou-Kou Wang, MD, Yiu-Wah Li, MD,” Ming-Lon Young, MD, Su-Wen How, MD,b and Hung-Chi Lue, MD Taipei, Taiwan From the Departments of Pediatrics, Taiwan University Hospital. Reprint Taiwan 4/4/387
requests: University 19
Jou-Kou Hospital,
Radiology,*
and Pathology,b
National
Wang, MD, Department of Pediatrics, National No. 1 Chang-Te St.. Taipei, Taiwan.
807
Patients with obstructed total anomalous pulmonary venous connection (TAPVC) usually have cardiac symptoms very early in 1ife.i Therefore detection of the presence of pulmonary venousobstruction is mandatory for optimal management. Recently ECG gated magnetic resonance imaging (MRI) has beenwidely usedto study various cardiovascular anomalies.2Results of a previous report3 showed that MRI could delineate the pulmonary venous obstruction that occurred after surgical correction of TAPVC. We utilized this technique to delineate the site of pulmonary venous obstruction in three infants with TAPVC, aspleniasyndrome, and complex cardiovascular anomalies.MRI studies were performed by means of a
Fig. 1. A, MRI coronal section (patient 1) showingpulmonary venousconfluence(PVC) ascendingto form vertical vein (VV) traversing pulmonary parenchyma. Small arrows indicate intrapulmonary segmentof vertical vein. B, Seriesof sagittal sections(patient 1) showingpathway of vertical vein. Junction of vertical
vein with SVC wasvery narrow. Right SVC wasaneurysmally dilated. C, Heart and lung specimen(patient 1, anterior view with heart reflected). Pulmonary venous confluence behind heart wasshown. Arrowhead indicates entry site of vertical vein into right upper lobe (RUL) of lung. Vertical vein then connected to right SVC via narrow segment (arrow). Right SVC was aneurysmally dilated.
808
Communications
Brief
Amman
Fig. 2. MRI coronal svc.
Table
I. A summary
section (patient
2) showing
of cardiovascular
anomalies
Age
Sex
1
2 days
F
2
2 wk
M
3
6 mo
M
Patient
no.
ASD, Atria.1 septal defect: AV, *Diagnosis of asplenia syndrome
atrioventricular; was made
DORV, on the basis
vein stenosis at point of entry (arrow)
into right
in three patients C’ardiouascular Asplenia syndrome,* ventricle, DORV, Asplenia syndrome.* with obstruction Asplenia syndrome,* ventricle. DORV,
double-outlet of high-kilovoltage
spin-echo technique with the use of a 1.5 Tesla (General Electric CGR USA, Milwaukee, Wis.) Tesla scanner (Hitachi Medical Corp. of America, town, N.Y.). Multiple sectiohs, with thicknesses from 3 to 5 mm, were acquired through various planes. A head coil was used in all patients.
vertical
September 1992 Heart Journal
right
scanner or a 0.2 Tarryranging imaging
anomalies
ASD, common AV valve, double-inlet right PS, TAPVC to right SVC with obstruction AV canal, DORV. PS. TAPVC to right SVC ASD, common AV valve, double-inlet PS, TAPVC to left SVC with obstruction
ventricle; PS. pulmonic chest film, a peripheral
stenosis. blood smear,
echocardiography,
angiopraphy,
right
and MRl.
Patient 1. A female newborn infant had cyanosis and tachypnea soon after birth. She was found to have complex heart disease with asplenia syndrome (Table I). On echocardiography, a pulmonary venous confluence with no connection to the atria ascended as a vertical vein. The vertical vein seemed to connect to the right superior vena
Volume
124
Number
3
cava (SVC) with severe obstruction where a turbulent flow could be recorded with an estimated gradient of 20 mm Hg. At cardiac catheterization, both pulmonary and digital subtraction angiography failed to demonstrate a pulmonary venous pathway. MRI studies revealed that the pulmonary venous confluence ascended to form a vertical vein, which traversed the pulmonary parenchyma and joined the right SVC via a narrow segment (Fig. 1,A and B). There was aneurysmal dilatation of the right SVC. The infant died of cardiac failure before undergoing surgery. These findings were confirmed at autopsy (Fig. 1, C). Patient 2. A 2-week-old male infant had cyanosis and tachypnea at birth. The cardiac anomalies are listed in Table I. Although it was not well delineated, the echocardiogram showed that the vertical vein was connected to the right SVC where a turbulent flow was recorded. The gradient estimated by the Bernoulli equation was 16 mm Hg. Digital subtraction angiography confirmed the presence of an obstruction at the site of pulmonary venous drainage. Coronal MRI revealed that the point of connection of the vertical vein was stenotic and the right SVC was aneurysmally dilated (Fig. 2). The patient died after surgery. The findings were confirmed at autopsy. Patient 3. A 6-month-old male infant was referred to this instit.ut,ion because of cyanosis and dyspnea since early infancy. A chest roentgenogram showed marked pulmonary venous congestion. The cardiovascular anomalies are summarized in Table I. A “membrane,” found at the junction of the pulmonary venous confluence with the left SVC, appeared to obstruct the pulmonary venous return. A prostaglandin challenge test resulted in severe dyspnea.4 Angiography failed to demonstrate a pulmonary venous pathway. MRI delineated the site of pulmonary venous obstruction (Fig. 3). An anastomosis of the pulmonary venous confluence to the atrium was performed successfully. It is known that 48’1 of TAPVCs in the asplenia syndrome have pulmonary venous obstruction.5 In two of three patients the site of pulmonary obstruction was difficult to image by cross-sectional echocardiography, but a turbulent flow appeared in the dilated SVC suggesting the presence of an obstruction to pulmonary venous return. In two patients angiography failed to delineate either the pulmonary venous pathway or the obstruction. In contrast, MRI clearly depicted these lesions in all three of our patients. By means of angiography, the pulmonary venous pathway is difficult to image in the presence of pulmonary venous obstruction.’ Both the poor echo window and the in patients complexity of the pulmonary venous system with asplenia syndrome make echocardiographic study difficult. Furthermore, decreased pulmonary blood flow caused by concomitant pulmonary stenosis or atresia, which commonly occurs in asplenia syndrome, could mask the presence of pulmonary venous obstruction.“, 6 On the other hand, an MRI study that could easily investigate a deep structure in the thoracic cavity and noninvasively provide spat.ial relationships of the vessels and cardiac cambers makes it a complementary imaging modality to echocardiography in the evaluation of TAPVC with obstruction in the asplenia syndrome. In one patient the ver-
Brief Communications
809
Fig. 3. MRI coronal section (patient 3) showing “membrane” (arrowhead) at junction of pulmonary venous confluence (PVC) with left SVC.
tical vein traversed the pulmonary parenchyma and connected with the right SVC. This finding, which was later proved at autopsy, is extremely rare. Aneurysmal dilatation of the SVC existed in two patients with a turbulent flow appearing in the SVC on color Doppler imaging (patients 1 and 2). The aneurysmal dilatation of the SVC could have resulted from the effect of the turbulent flow coming from the obstructed pulmonary venous return.
REFERENCES
1. DeLisle G, Ando M, Calder AL, et al. Total anomalous pulmonary venous connection: report of 93 autopsied cases with emphasis on diagnostic and surgical considerations. AM HEAKT d 1976;91:99-122. 2. Didier D, Higgins CB, Fisher MR, Osaki L, Silverman NH, Cheitlin MD. Congenital heart disease: gated MR imaging in 72 patients. Radiology 1986;158:227-35. 3. Ross RD, Bisset GS, Meyer RA, Hannon DW, Bove KE. Magnetic resonance imaging for diagnosis of pulmonary vein stenosis after correction of total anomalous pulmonary venous connection. Am J Cardiol 1987;60:1199-201. 4. Freedom RM, Olley PM, Coceani F. Rowe RD. The prostaglandin challenge test to unmask obstructed total anomalous pulmonary connection in asplenia syndrome. Br Heart J 1978;40:91-4. 5. Gathman GE, Nadas AS. Total anomalous pulmonary venous connection. Clinical and physiologic observation of 75 pediatric patients. Circulation 1970;42:143-54. 6. Rose V, Izukawa T, Moes CAF. Syndrome of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases with special reference to diagnosis and prognosis. Br Heart d 1975:X7:840-52.
124
Number
3
Brief Communications
Delineation of obstruction in total anomalous pulmonary venous connection utilizing magnetic resonance imaging Jou-Kou Wang, MD, Yiu-Wah Li, MD,” Ming-Lon Young, MD, Su-Wen How, MD,b and Hung-Chi Lue, MD Taipei, Taiwan From the Departments of Pediatrics, Taiwan University Hospital. Reprint Taiwan 4/4/387
requests: University 19
Jou-Kou Hospital,
Radiology,*
and Pathology,b
National
Wang, MD, Department of Pediatrics, National No. 1 Chang-Te St.. Taipei, Taiwan.
807
Patients with obstructed total anomalous pulmonary venous connection (TAPVC) usually have cardiac symptoms very early in 1ife.i Therefore detection of the presence of pulmonary venousobstruction is mandatory for optimal management. Recently ECG gated magnetic resonance imaging (MRI) has beenwidely usedto study various cardiovascular anomalies.2Results of a previous report3 showed that MRI could delineate the pulmonary venous obstruction that occurred after surgical correction of TAPVC. We utilized this technique to delineate the site of pulmonary venous obstruction in three infants with TAPVC, aspleniasyndrome, and complex cardiovascular anomalies.MRI studies were performed by means of a
Fig. 1. A, MRI coronal section (patient 1) showingpulmonary venousconfluence(PVC) ascendingto form vertical vein (VV) traversing pulmonary parenchyma. Small arrows indicate intrapulmonary segmentof vertical vein. B, Seriesof sagittal sections(patient 1) showingpathway of vertical vein. Junction of vertical
vein with SVC wasvery narrow. Right SVC wasaneurysmally dilated. C, Heart and lung specimen(patient 1, anterior view with heart reflected). Pulmonary venous confluence behind heart wasshown. Arrowhead indicates entry site of vertical vein into right upper lobe (RUL) of lung. Vertical vein then connected to right SVC via narrow segment (arrow). Right SVC was aneurysmally dilated.
808
Communications
Brief
Amman
Fig. 2. MRI coronal svc.
Table
I. A summary
section (patient
2) showing
of cardiovascular
anomalies
Age
Sex
1
2 days
F
2
2 wk
M
3
6 mo
M
Patient
no.
ASD, Atria.1 septal defect: AV, *Diagnosis of asplenia syndrome
atrioventricular; was made
DORV, on the basis
vein stenosis at point of entry (arrow)
into right
in three patients C’ardiouascular Asplenia syndrome,* ventricle, DORV, Asplenia syndrome.* with obstruction Asplenia syndrome,* ventricle. DORV,
double-outlet of high-kilovoltage
spin-echo technique with the use of a 1.5 Tesla (General Electric CGR USA, Milwaukee, Wis.) Tesla scanner (Hitachi Medical Corp. of America, town, N.Y.). Multiple sectiohs, with thicknesses from 3 to 5 mm, were acquired through various planes. A head coil was used in all patients.
vertical
September 1992 Heart Journal
right
scanner or a 0.2 Tarryranging imaging
anomalies
ASD, common AV valve, double-inlet right PS, TAPVC to right SVC with obstruction AV canal, DORV. PS. TAPVC to right SVC ASD, common AV valve, double-inlet PS, TAPVC to left SVC with obstruction
ventricle; PS. pulmonic chest film, a peripheral
stenosis. blood smear,
echocardiography,
angiopraphy,
right
and MRl.
Patient 1. A female newborn infant had cyanosis and tachypnea soon after birth. She was found to have complex heart disease with asplenia syndrome (Table I). On echocardiography, a pulmonary venous confluence with no connection to the atria ascended as a vertical vein. The vertical vein seemed to connect to the right superior vena
Volume
124
Number
3
cava (SVC) with severe obstruction where a turbulent flow could be recorded with an estimated gradient of 20 mm Hg. At cardiac catheterization, both pulmonary and digital subtraction angiography failed to demonstrate a pulmonary venous pathway. MRI studies revealed that the pulmonary venous confluence ascended to form a vertical vein, which traversed the pulmonary parenchyma and joined the right SVC via a narrow segment (Fig. 1,A and B). There was aneurysmal dilatation of the right SVC. The infant died of cardiac failure before undergoing surgery. These findings were confirmed at autopsy (Fig. 1, C). Patient 2. A 2-week-old male infant had cyanosis and tachypnea at birth. The cardiac anomalies are listed in Table I. Although it was not well delineated, the echocardiogram showed that the vertical vein was connected to the right SVC where a turbulent flow was recorded. The gradient estimated by the Bernoulli equation was 16 mm Hg. Digital subtraction angiography confirmed the presence of an obstruction at the site of pulmonary venous drainage. Coronal MRI revealed that the point of connection of the vertical vein was stenotic and the right SVC was aneurysmally dilated (Fig. 2). The patient died after surgery. The findings were confirmed at autopsy. Patient 3. A 6-month-old male infant was referred to this instit.ut,ion because of cyanosis and dyspnea since early infancy. A chest roentgenogram showed marked pulmonary venous congestion. The cardiovascular anomalies are summarized in Table I. A “membrane,” found at the junction of the pulmonary venous confluence with the left SVC, appeared to obstruct the pulmonary venous return. A prostaglandin challenge test resulted in severe dyspnea.4 Angiography failed to demonstrate a pulmonary venous pathway. MRI delineated the site of pulmonary venous obstruction (Fig. 3). An anastomosis of the pulmonary venous confluence to the atrium was performed successfully. It is known that 48’1 of TAPVCs in the asplenia syndrome have pulmonary venous obstruction.5 In two of three patients the site of pulmonary obstruction was difficult to image by cross-sectional echocardiography, but a turbulent flow appeared in the dilated SVC suggesting the presence of an obstruction to pulmonary venous return. In two patients angiography failed to delineate either the pulmonary venous pathway or the obstruction. In contrast, MRI clearly depicted these lesions in all three of our patients. By means of angiography, the pulmonary venous pathway is difficult to image in the presence of pulmonary venous obstruction.’ Both the poor echo window and the in patients complexity of the pulmonary venous system with asplenia syndrome make echocardiographic study difficult. Furthermore, decreased pulmonary blood flow caused by concomitant pulmonary stenosis or atresia, which commonly occurs in asplenia syndrome, could mask the presence of pulmonary venous obstruction.“, 6 On the other hand, an MRI study that could easily investigate a deep structure in the thoracic cavity and noninvasively provide spat.ial relationships of the vessels and cardiac cambers makes it a complementary imaging modality to echocardiography in the evaluation of TAPVC with obstruction in the asplenia syndrome. In one patient the ver-
Brief Communications
809
Fig. 3. MRI coronal section (patient 3) showing “membrane” (arrowhead) at junction of pulmonary venous confluence (PVC) with left SVC.
tical vein traversed the pulmonary parenchyma and connected with the right SVC. This finding, which was later proved at autopsy, is extremely rare. Aneurysmal dilatation of the SVC existed in two patients with a turbulent flow appearing in the SVC on color Doppler imaging (patients 1 and 2). The aneurysmal dilatation of the SVC could have resulted from the effect of the turbulent flow coming from the obstructed pulmonary venous return.
REFERENCES
1. DeLisle G, Ando M, Calder AL, et al. Total anomalous pulmonary venous connection: report of 93 autopsied cases with emphasis on diagnostic and surgical considerations. AM HEAKT d 1976;91:99-122. 2. Didier D, Higgins CB, Fisher MR, Osaki L, Silverman NH, Cheitlin MD. Congenital heart disease: gated MR imaging in 72 patients. Radiology 1986;158:227-35. 3. Ross RD, Bisset GS, Meyer RA, Hannon DW, Bove KE. Magnetic resonance imaging for diagnosis of pulmonary vein stenosis after correction of total anomalous pulmonary venous connection. Am J Cardiol 1987;60:1199-201. 4. Freedom RM, Olley PM, Coceani F. Rowe RD. The prostaglandin challenge test to unmask obstructed total anomalous pulmonary connection in asplenia syndrome. Br Heart J 1978;40:91-4. 5. Gathman GE, Nadas AS. Total anomalous pulmonary venous connection. Clinical and physiologic observation of 75 pediatric patients. Circulation 1970;42:143-54. 6. Rose V, Izukawa T, Moes CAF. Syndrome of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases with special reference to diagnosis and prognosis. Br Heart d 1975:X7:840-52.
