Ann Thorac Surg 1996;62:1517-1519
© 1996 The Society of Thoracic Surgeons
Case Report
Repair of an Unusual Type of Total Anomalous Pulmonary Venous Connection
Kevin W. Lobdell, MD,
Henry L. Walters, III, MD,
Vijaya Joshi, MD,
Robert D. Ross, MD,
Mehdi Hakimi, MD
Departments of Cardiovascular Surgery and Cardiology, Children's Hospital of Michigan, Detroit, Michigan
Accepted for publication May 14, 1996.
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Abstract
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A 9-day-old, 4.5-kg female infant was diagnosed with total anomalous pulmonary venous connections by echocardiography. The unusual nature of the pulmonary veins and their connections led to cardiac catheterization and angiography. Angiography demonstrated the left-sided veins connected, via a retropulmonary artery vertical vein, to the left inominate vein whereas the right-sided pulmonary veins connected to the right superior vena cava. We repaired this lesion uneventfully by creating a neo-common pulmonary vein and anastomosing this confluence to the left atrium.
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Introduction
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Abnormal development of the common pulmonary vein is implicated as the embryologic basis for total anomalous pulmonary venous connections. The evolution of diagnostic procedures, surgical techniques, and perioperative management has rendered this previously vexing lesion more approachable, resulting in low operative mortality and excellent long-term survival [1–3]. Anatomic classification of isolated total anomalous pulmonary venous connections includes four types [4]. Rarely, a group, which defies categorization with the aforementioned scheme, may exhibit dual systemic connections. This rare group possesses a confluence (common pulmonary vein) with two connections to the systemic circulation [5].
A 9-day-old, 4.5-kg female infant was hospitalized with vomiting, tachypnea, and cyanosis and then transferred to Children's Hospital of Michigan. Progressive tachypnea and cyanosis resulted in support with positive-pressure mechanical ventilation. Physical examination was significant for a 2/6 systolic murmur. Arterial blood gas analysis revealed a pH of 7.38, carbon dioxide tension of 44 mm Hg, and oxygen tension of 37 mm Hg (inspired oxygen fraction = 1.0, rate = 30 breaths/min, peak inspiratory pressure = 30 cm H2O, and positive end-expiratory pressure = 4 cm H2O). Chest radiography demonstrated a moderately enlarged cardiac silhouette and a reticular pulmonary pattern consistent with mild to moderate pulmonary venous congestion. Echocardiography demonstrated tortuous left pulmonary veins connecting to the left inominate vein, via a vertical vein coursing between the left pulmonary artery and bronchus. The right-sided pulmonary veins were tortuous but the relationship to the left-sided pulmonary veins and systemic venous system could not be ascertained. Doppler analysis of mild tricuspid valve regurgitation estimated right ventricular pressure to be suprasystemic. The remainder of the echocardiogram was characteristic with right to left bowing of the interatrial septum, a small defect beneath the superior limbic band, and a diminutive left atrium. A ductus arteriosus was present and demonstrated bidirectional flow. Cardiac angiography and pressure measurements were obtained because of the unusual nature of the connections and serpiginous course of the pulmonary veins.
Angiography (Fig 1
) corroborated the serpiginous route of the pulmonary veins and demonstrated the left-sided connection to the left inominate vein whereas the right-sided veins connected to the right superior vena cava. The right ventricular pressure was 78 mm Hg, whereas the aortic pressure was 62 mm Hg. The obstruction to the left-sided pulmonary veins resulted in 14 mm Hg gradient across the left vertical vein's course between the left pulmonary artery and the left bronchus.
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Fig 1. . Angiogram of total anomalous pulmonary venous connections without a common pulmonary vein. The left pulmonary veins connect to the left inominate vein via a vertical vein and the right pulmonary veins connect to the right superior vena cava.
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Emergent repair was performed via median sternotomy with the aid of deep hypothermia and circulatory arrest while myocardial protection was provided by intermitent, hypothermic, hyperkalemic blood cardioplegia and topical iced saline solution. Thorough dissection permitted approximation of the right and left pulmonary veins. A transverse incision was made in the right atrium and carried across the interatrial septum to the base of the left atrial appendage. A "neo-common" pulmonary vein was fashioned by creating a longitudinal venotomy in both the right and left pulmonary veins and then approximating the juxtaposed veins with a continuous 7-0 polypropylene suture. (The longitudinal venotomy was slightly longer on the right pulmonary vein than that on the left.) The newly created pulmonary venous confluence was anastomosed to the posterior aspect of the left atrium (Fig 2). The anomalous connections were not ligated to allow "pop-off" in the event of a pulmonary hypertensive crisis.
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Fig 2. . Creation of a neo-common pulmonary vein from separate right and left pulmonary veins and anastomosis to the left atrium via a linear biatrial incision.
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The patient's postoperative course was remarkable for severe pulmonary hypertension, treated effectively with nitric oxide after failure of conventional regimens. Satisfactory repair was ascertained 5 months after correction.
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Comment
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The case we describe is a rare lesion-dual supracardiac connections without a common pulmonary vein and obstruction-demonstrating the variety of characteristics that one may encounter in total anomalous pulmonary venous connections, but in extraordinary combination. We applied a surgical strategy reminiscent of standard techniques [6, 7] and with the creation of a neo-common pulmonary vein. The postoperative course was typical of a patient presenting with obstructed total anomalous pulmonary venous connections and included the use of inhaled nitric oxide for refractory pulmonary hypertension.
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Footnotes
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Address reprint requests to Dr Lobdell, Department of Cardiovascular Surgery, Children's Hospital of Michigan, 3901 Beaubien Blvd, Detroit, MI 48201.
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References
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- Muller WH. The surgical treatment of transposition of the pulmonary veins. Ann Surg 1951;134:683–93.
- Lewis FJ, Varco RL, Taufic M, Niazi MB. Direct vision repair of triatrial heart and total anomalous pulmonary venous drainage. Surg Gynecol Obstet 1956;102:713–20.[Medline]
- Gott VL, Lester RG, Lillehei CW, Varco RL. Total anomalous pulmonary venous return. An analysis of thirty cases. Circulation 1956;13:543–52.[Medline]
- Darling RC, Rothney WB, Craig JM. Total anomalous venous drainage to the right side of the heart. Lab Invest 1957;6:44–64.[Medline]
- Arciprete P, McKay R, Watson GH, Hamilton DI, Wilkinson JL, Arnold RM. Double connections in total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg 1986;92:146–52.[Abstract]
- Kirklin JW, Barratt-Boyes BG. Total anomalous pulmonary venous connection. In: Kirklin JW, Barratt-Boyes BG, eds. Cardiac surgery. Second ed. New York: Churchill Livingstone, 1993:645–73.
- Castañeda AR, Jonas RA, Mayer JE, Hanley FL. Anomalies of the pulmonary veins. In: Cardiac surgery of the neonate and infant. Philadelphia: Saunders, 1994;157–66.
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Abstract
Introduction
