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Surgical repair of inferior sinus venosus atrial septal defect
J THORAC CARDIOVASC SURG 78:570-572, 1979
Surgical repair of inferior sinus venosus atrial septal defect A 9-year-old girl presented for cardiac evaluation with symptoms of dyspena, fatigue, and cyanosis with exercise. Cardiac catheterization demonstrated an atrial septal defect; an anomalous right superior pulmonary vein was suspected but not confirmed. Operation disclosed anomalous drainage of the right superior and inferior pulmonary veins into the right atrium, an intact fossa ovalis, and an inferior sinus venosus defect. Repair was accomplished by detaching the posterior edge of the atrial septum and suturing it to the right of the pulmonary veins, so that the defect was closed and all the pulmonary venous blood was directed to the left atrium.
James T. Sturm, M.D., and Jay L. Ankeney, M.D., Cleveland, Ohio
Atrial septal defect constitutes one of the most common types of congenital heart disease. Three common varieties of this anomaly include ostium secundum, ostium primum, and superior sinus venosus types. Partial anomalous pulmonary venous connection usually accompanies superior sinus venosus defects; in such cases the septal defect is high and in proximity to the superior vena cava-right atrial junction. Usually the superior pulmonary veins from the upper lobe of the right lung drain into the superior vena cava. This report describes a case of inferior sinus venosus defect with anomalous connection of both the superior and inferior pulmonary veins to the right atrium, and illustrates a method of surgical correction.
Case report A 9-year-old girl was admitted for elective cardiac catheterization. She was the first born of twins and weighed 2.4 kg at birth. Born 3 weeks prematurely she was slightly cyanotic and failed to breathe spontaneously at first, but was resuscitated uneventfully. She was healthy as a neonate but was hospitalized for pneumonia at the age of 28 months. A murmur had been detected but not followed when she was 19 months old. The child did well until the age of 9 years, when she was referred for cardiological evaluation because of symptoms of dyspnea, fatigue, and slight cyanosis with exercise. From the Division of Cardiothoracic Surgery, University Hospitals, Cleveland, Ohio 44106. Received for publication March 29, 1979. Accepted for publication May 8, 1979. Address for reprints: Dr. Jay L. Ankeney, Division of CardioThoracic Surgery, University Hospitals, 2065 Adelbert Rd., Cleveland, Ohio 44106.
570
Abnormal physical findings were confined to the cardiac examination. Cardiac activity was mildly hyperdynamic to palpation. The second heart sound was widely split and fixed. A Grade 2/6 systolic ejection murmur was auscultated at the upper left sternal border and a mid-diastolic rumble was heard at the lower left sternal border. There was no third or fourth heart sound. The chest roentgenogram demonstrated increased pulmonary vascular markings and a large main pulmonary artery. The electrocardiogram showed incomplete right bundle branch block, right ventricular hypertrophy, and right axis deviation. Cardiac catheterization demonstrated an atrial septal defect with a left-to-right shunt. The pulmonary-to-systemic flow ratio was calculated to be 3: I with normal right ventricular and pulmonary artery pressures, suggesting low pulmonary vascular resistance. An anomalous right superior pulmonary vein was suspected but not confirmed. The patient was referred to our hospital for surgical correction of the atrial septal defect. Operation was performed on Oct. 31, 1977. A right inframammary incision was made through the bed of the unresected fourth rib. When the pericardium was opened, the right superior and right inferior pulmonary veins were noted to drain into the right atrium. Cardiopulmonary bypass was established and the right atrium opened. Inspection disclosed an intact fossa ovalis, a normal tricuspid valve, and a normal coronary sinus. The atrial septal defect was 3 ern in its longest dimension and 2 em in the anteroposterior axis. The posterior margin of the defect was formed by the posterior right atrial wall rather than by a portion of septum. The right superior pulmonary vein entered the right atrium cephalad to the defect, and the right inferior pulmonary vein entered opposite the defect (Fig. 1). Direct suture repair of the defect was not possible without excluding the right superior pulmonary vein from the left atrium. Accordingly, the posterior atrial septum was detached from the level of the septal defect to I cm cephalad to the opening of the right superior pulmonary vein (Fig. 2). Next, the anterior edge of the septal defect and the edge of the detached septum were sutured to the right of the pulmonary veins with interrupted figure-of-eight 3-0 silk su-
0022-5223/79/100570+03$00.30/0 © 1979 The C. V. Mosby Co.
Volume 78 Number 4
Inferior sinus venosus atrial septal defect
October, 1979
57 I
PV _ _-«
'~"rlJ~-
__
..."II~---_T
V
SD
D_ _................
CD Fig. 1. Entry of both the right superior and right inferior pulmonary veins (PV) into the right atrium, the low atrial septal defect (SD) and the tricuspid valve (TV). Fig. 2. Detachment (D) of the posterior atrial septum. Fig. 3. Line of closure (C). The anterior edge of the atrial septal defect and the anterior margin of the septal detachment have been transposed to the right of the pulmonary veins. tures. The pulmonary venous drainage was thereby directed to the left atrium without compromising the orifices of the coronary sinus, superior vena cava, or inferior vena cave (Fig. 3). The atriotomy was closed, cardiopulmonary bypass was terminated without difficulty, and the chest was closed. The patient made an uneventful recovery and is doing well 18 months following the operation.
Discussion As many as 23% of atrial septal defects encountered at operation may be located low in the septum, and one
margin of the defect may be formed by the right atrial wall rather than by a portion of septum. 1 The defect in the present case was not a variation of the secundum type because the fossa ovalis was present and intact. The association of an inferior sinus venosus defect with drainage of both the superior and inferior pulmonary veins into the right atrium is a distinct rarity.f Superior sinus venosus defects result from malposition of the orifice of the superior sinus venosus and overmigration of the pulmonary veins. 3 Maldevelopment of the in-
The Journal of
572
Sturm and Ankeney
Thoracic and Cardiovascular Surgery
ferior sinus venosus and ovennigration of both superior and inferior pulmonary veins probably resulted in the inferior sinus venosus defect herein reported. 4 Two anatomic points must be kept in mind when repairing this type of defect. Care must be taken to divert flow from both anomalous pulmonary veins into the left atrium. When dealing with low-lying atrial septal defects, caution should be exercised so that the eusthachian valve is not mistaken for the lower margin of the defect. If the eustachian valve is mistakenly incorporated into the suture line of the repair, inferior vena caval blood will be diverted to the left atrium.
REFERENCES Sellors TH: Atrial septal defects. Ann R Coli Surg Engl 46:1-19, 1970 2 McConnack RJM, Pickering D: A rare type of atrial septal defect. Thorax 23:350-352, 1968 3 Harley HRS: The sinus venosus type of atrial septal defect. Thorax 13:12-27, 1958 4 Goor DA, Lillehei CW: Congenital Malformations of the Heart, New York, 1975, Grune & Stratton, Inc.. p 106
Surgical repair of inferior sinus venosus atrial septal defect A 9-year-old girl presented for cardiac evaluation with symptoms of dyspena, fatigue, and cyanosis with exercise. Cardiac catheterization demonstrated an atrial septal defect; an anomalous right superior pulmonary vein was suspected but not confirmed. Operation disclosed anomalous drainage of the right superior and inferior pulmonary veins into the right atrium, an intact fossa ovalis, and an inferior sinus venosus defect. Repair was accomplished by detaching the posterior edge of the atrial septum and suturing it to the right of the pulmonary veins, so that the defect was closed and all the pulmonary venous blood was directed to the left atrium.
James T. Sturm, M.D., and Jay L. Ankeney, M.D., Cleveland, Ohio
Atrial septal defect constitutes one of the most common types of congenital heart disease. Three common varieties of this anomaly include ostium secundum, ostium primum, and superior sinus venosus types. Partial anomalous pulmonary venous connection usually accompanies superior sinus venosus defects; in such cases the septal defect is high and in proximity to the superior vena cava-right atrial junction. Usually the superior pulmonary veins from the upper lobe of the right lung drain into the superior vena cava. This report describes a case of inferior sinus venosus defect with anomalous connection of both the superior and inferior pulmonary veins to the right atrium, and illustrates a method of surgical correction.
Case report A 9-year-old girl was admitted for elective cardiac catheterization. She was the first born of twins and weighed 2.4 kg at birth. Born 3 weeks prematurely she was slightly cyanotic and failed to breathe spontaneously at first, but was resuscitated uneventfully. She was healthy as a neonate but was hospitalized for pneumonia at the age of 28 months. A murmur had been detected but not followed when she was 19 months old. The child did well until the age of 9 years, when she was referred for cardiological evaluation because of symptoms of dyspnea, fatigue, and slight cyanosis with exercise. From the Division of Cardiothoracic Surgery, University Hospitals, Cleveland, Ohio 44106. Received for publication March 29, 1979. Accepted for publication May 8, 1979. Address for reprints: Dr. Jay L. Ankeney, Division of CardioThoracic Surgery, University Hospitals, 2065 Adelbert Rd., Cleveland, Ohio 44106.
570
Abnormal physical findings were confined to the cardiac examination. Cardiac activity was mildly hyperdynamic to palpation. The second heart sound was widely split and fixed. A Grade 2/6 systolic ejection murmur was auscultated at the upper left sternal border and a mid-diastolic rumble was heard at the lower left sternal border. There was no third or fourth heart sound. The chest roentgenogram demonstrated increased pulmonary vascular markings and a large main pulmonary artery. The electrocardiogram showed incomplete right bundle branch block, right ventricular hypertrophy, and right axis deviation. Cardiac catheterization demonstrated an atrial septal defect with a left-to-right shunt. The pulmonary-to-systemic flow ratio was calculated to be 3: I with normal right ventricular and pulmonary artery pressures, suggesting low pulmonary vascular resistance. An anomalous right superior pulmonary vein was suspected but not confirmed. The patient was referred to our hospital for surgical correction of the atrial septal defect. Operation was performed on Oct. 31, 1977. A right inframammary incision was made through the bed of the unresected fourth rib. When the pericardium was opened, the right superior and right inferior pulmonary veins were noted to drain into the right atrium. Cardiopulmonary bypass was established and the right atrium opened. Inspection disclosed an intact fossa ovalis, a normal tricuspid valve, and a normal coronary sinus. The atrial septal defect was 3 ern in its longest dimension and 2 em in the anteroposterior axis. The posterior margin of the defect was formed by the posterior right atrial wall rather than by a portion of septum. The right superior pulmonary vein entered the right atrium cephalad to the defect, and the right inferior pulmonary vein entered opposite the defect (Fig. 1). Direct suture repair of the defect was not possible without excluding the right superior pulmonary vein from the left atrium. Accordingly, the posterior atrial septum was detached from the level of the septal defect to I cm cephalad to the opening of the right superior pulmonary vein (Fig. 2). Next, the anterior edge of the septal defect and the edge of the detached septum were sutured to the right of the pulmonary veins with interrupted figure-of-eight 3-0 silk su-
0022-5223/79/100570+03$00.30/0 © 1979 The C. V. Mosby Co.
Volume 78 Number 4
Inferior sinus venosus atrial septal defect
October, 1979
57 I
PV _ _-«
'~"rlJ~-
__
..."II~---_T
V
SD
D_ _................
CD Fig. 1. Entry of both the right superior and right inferior pulmonary veins (PV) into the right atrium, the low atrial septal defect (SD) and the tricuspid valve (TV). Fig. 2. Detachment (D) of the posterior atrial septum. Fig. 3. Line of closure (C). The anterior edge of the atrial septal defect and the anterior margin of the septal detachment have been transposed to the right of the pulmonary veins. tures. The pulmonary venous drainage was thereby directed to the left atrium without compromising the orifices of the coronary sinus, superior vena cava, or inferior vena cave (Fig. 3). The atriotomy was closed, cardiopulmonary bypass was terminated without difficulty, and the chest was closed. The patient made an uneventful recovery and is doing well 18 months following the operation.
Discussion As many as 23% of atrial septal defects encountered at operation may be located low in the septum, and one
margin of the defect may be formed by the right atrial wall rather than by a portion of septum. 1 The defect in the present case was not a variation of the secundum type because the fossa ovalis was present and intact. The association of an inferior sinus venosus defect with drainage of both the superior and inferior pulmonary veins into the right atrium is a distinct rarity.f Superior sinus venosus defects result from malposition of the orifice of the superior sinus venosus and overmigration of the pulmonary veins. 3 Maldevelopment of the in-
The Journal of
572
Sturm and Ankeney
Thoracic and Cardiovascular Surgery
ferior sinus venosus and ovennigration of both superior and inferior pulmonary veins probably resulted in the inferior sinus venosus defect herein reported. 4 Two anatomic points must be kept in mind when repairing this type of defect. Care must be taken to divert flow from both anomalous pulmonary veins into the left atrium. When dealing with low-lying atrial septal defects, caution should be exercised so that the eusthachian valve is not mistaken for the lower margin of the defect. If the eustachian valve is mistakenly incorporated into the suture line of the repair, inferior vena caval blood will be diverted to the left atrium.
REFERENCES Sellors TH: Atrial septal defects. Ann R Coli Surg Engl 46:1-19, 1970 2 McConnack RJM, Pickering D: A rare type of atrial septal defect. Thorax 23:350-352, 1968 3 Harley HRS: The sinus venosus type of atrial septal defect. Thorax 13:12-27, 1958 4 Goor DA, Lillehei CW: Congenital Malformations of the Heart, New York, 1975, Grune & Stratton, Inc.. p 106