Coronary artery fistula into a persistent left superior vena cava: Report of a case

We herein report the rare case of a patient with coronary artery fistula (CAF) between the left circumflex coronary artery and persistent left superior vena cava (PLSVC) with a complete absence of the right superior vena cava (SVC).     

Anomalies of the Systemic Venous Return: A Review

Congenital anomalies of the systemic venous connection to the heart represent a rather wide and heterogeneous group of malformations, whose physiological consequences may vary from nil to the most severe form of systemic arterial desaturation. The malformations may be summarized as follows: (1) Left superior vena cava connected to the coronary sinus, interrupted inferior vena cava and absent right superior vena cava that do not indicate surgical repair 'per se', but require some technical attention during open heart surgery performed for other anomalies; (2) Left superior vena cava connected to the left atrium, due to incorporation of the coronary sinus into the left atrial cavity, resulting in a right-to-left-shunt; (3) Right superior vena cava or inferior vena cava draining into the left atrium, both are extremely rare and require treatment for the ensuing right-to-left shunt; (4) Total anomalous systemic venous connection to the left atrium, usually combined with atrial isomerism and other very complex heart malformations; (5) Cor triatriatum dexter, which has been frequently diagnosed as an anomalous venous connection for its similar hemodynamic consequences. Such anomalies are reviewed with particular respect to their surgical implications.     

Anomalous dual drainage of the right pulmonary veins in a patient with cor triatriatum: report of a case without scimitar sign

The majority of patients with partial anomalous drainage of pulmonary veins are asymptomatic during infancy and childhood. Patients with significant left-to-right shunt develop symptoms and benefit from early corrective surgery. Anomalous pulmonary veins draining into inferior vena cava is very rare and frequently encountered in association with scimitar syndrome. The purpose of this case report is to describe a non-scimitar patient with cor triatriatum who had anomalous dual drainage of right pulmonary veins into inferior vena cava/left atrium and anomalous connection of persistent left superior vena cava with a common pulmonary venous chamber. The patient underwent an operation with redirection of anomalous pulmonary venous drainage into left atrium and ligation of persistent left superior vena cava.     

Repair of lacerated intrapericardial inferior vena cava after cardiac massage

We successfully undertook surgical treatment of intrapericardial laceration of the inferior vena cava caused by external cardiac massage in a patient with acute coronary syndrome. Injury to the inferior vena cava without blunt trauma is very rare, and diagnostic imaging does not show it clearly, making it difficult to diagnose. Rapid and accurate judgment and management are necessary, because the mortality due to this injury is very high.     

Hemodynamic performance in a heterotopically transplanted dog heart: proposal of techniques for working left heart model of heterotopic (abdominal) heart transplantation.

To study hemodynamics together with various aspects of rejection after experimental heart transplantation, we developed a technique to produce a working left heart model of heterotopic (abdominal) heart transplantation. The interatrial septum and tricuspid valve of the donor heart are removed. The pulmonary arterial trunk, pulmonary veins, and inferior vena cava are ligated, and the stumps of the donor aorta and superior vena cava are anastomosed in an end-to-side fashion to the recipient abdominal aorta and inferior vena cava, respectively. Arterial blood from the recipient abdominal aorta thus perfuses the donor myocardium through the coronary artery, and the donor left ventricle receives venous blood from the recipient inferior vena cava as preload. In this model, the donor left ventricle does not pump out enough venous blood to desaturate the recipient femoral arterial blood but does generate approximately the same pressure as the recipient's heart. This model is reproducible, easy to manage, and can be applied to heterotopic heart transplantation in various experimental animals including rats.     

Bicaval anastomosis in a heart transplant recipient with left superior vena cava

We describe the surgical procedure of orthotopic heart transplantation (OHT) in a recipient with persistent left superior vena cava (LSVC) and isolated noncompaction of the left ventricle. The bicaval anastomosis technique was performed using and isolating his native coronary sinus to let the left superior vena cava drain into his own inferior vena cava through the native coronary sinus.     

Modified Total Cavopulmonary Connection: An Alternative Approach in the Presence of Left Superior Vena Cava

A technical modification to the total cavopulmonary connection in the presence of left superior vena cava (LSVC) is described. Systemic venous to pulmonary artery continuity is achieved by direct anastomosis of the right superior vena cava (RSVC) to the right pulmonary artery. Blood from the inferior vena cava (IVC) is diverted by an intraatrial baffle to the coronary sinus. The LSVC is then anastomosed to either the left pulmonary artery or the main pulmonary artery in an end-to-side or side-to-side manner. By using this technique, we maintain the basic principle of excluding the right atrial chamber from the systemic venous circuit, thereby reducing the potential obstructive complications that have been noted with other forms of complex intraatrial baffles. We have used this technique successfully in three patients with various forms of complex congenital cardiac defects.     

Left preureteral vena cava (retrocaval or circumcaval ureter) associated with partial situs inversus

We present a rare case of left preureteral vena cava associated with partial situs inversus. A 68-year-old woman was referred to our clinic for further study of left hydronephrosis on computerized tomography. The abdominal viscera were in mirror image and the heart was levocardia. The middle portion of the left ureter was dorsal to the left inferior vena cava. The left ureter was reanastomosed ventral to the inferior vena cava. To our knowledge, coexistence of preureteral vena cava and partial situs inversus has not been reported previously in the literature.     

Entrapment of pulmonary artery catheter in a suture at the inferior vena cava cannulation site

Entrapment of a pulmonary artery catheter (Swan-Ganz catheter) in the heart, vena cava, or pulmonary artery is a very rare and serious complication that may lead to life-threatening complications such as cardiac rupture, pulmonary artery rupture, cardiac tamponade, among others, if not recognized and treated early. We report entrapment of a Swan-Ganz catheter in the purse-string suture at the inferior vena cava cannulation site for a patient undergoing aortic valve replacement. This situation required a repeat sternotomy to release the pulmonary artery catheter.     

Hydatid cyst of the pulmonary artery secondary to hepatic hydatid cyst embolism

A cystic lesion of the liver extending into the inferior vena cava was discovered in a 33-year old female patient suffering from dyspnea, pain and swelling in the legs. Plain chest X-ray was normal. CT and MRI of the thorax showed a cystic lesion within the right pulmonary artery. The liver lesion was treated by a partial cystectomy and omentoplasty, inferior vena cava and bile ducts repair. Through a right thoracotomy, a hydatid cyst was found in the pulmonary artery and enucleated. Although very rare, pulmonary artery hydatidosis may be the cause of an unexplained dyspnea in patients with hydatidosis of the liver.     

Hydatid Cyst of the Pulmonary Artery Secondary to Hepatic Hydatid Cyst Embolism

A cystic lesion of the liver extending into the inferior vena cava was discovered in a 33-year old female patient suffering from dyspnea, pain and swelling in the legs. Plain chest X-ray was normal. CT and MRI of the thorax showed a cystic lesion within the right pulmonary artery. The liver lesion was treated by a partial cystectomy and omentoplas-ty, inferior vena cava and bile ducts repair. Through a right thoracotomy, a hydatid cyst was found in the pulmonary artery and enucleated. Although very rare, pulmonary artery hydatidosis may be the cause of an unexplained dyspnea in patients with hydatidosis of the liver.     

Coronary artery bypass grafting with absent right superior vena cava in visceroatrial situs solitus

We report on a 63-year-old man with an absence of right superior vena cava in visceroatrial situs solitus who underwent coronary artery bypass grafting. Preoperative echocardiography showed a dilated coronary sinus, and venography confirmed an absent right and a persistent left superior vena cava. Perioperatively, placement of a pulmonary artery catheter, site of venous cannulation, and management of associated rhythm abnormalities were of great concern. The assessment of the right superior vena cava is advisable in carrying out the surgical procedure without any difficulties related to this anomaly when the persistent left superior vena cava is suspected.     

High anomalous origin of both coronary arteries in a patient with aortic valve disease

High anomalous origin of both coronary arteries is extremely rare. We report the case of a patient with a right coronary artery that originated from the left surface of the ascending aorta approximately 25 mm above the sinotubular junction and a left coronary artery that originated from the sinotubular junction close to the non-left commissure. The patient also had persistent left superior vena cava. We diagnosed the anomaly preoperatively using 64-slice multidetector computed tomographic angiography. The detailed imaging information helped us to avoid coronary artery injury and perform the operation safely with adequate myocardial protection.     

Mechanical support of total cavopulmonary connection with an axial flow pump

OBJECTIVE: Even under optimal circumstances, total cavopulmonary connection is associated with a continuous late risk of death. Hemodynamics are distinctly abnormal, with increased systemic venous pressures and frequent low cardiac output. Our study uses a sheep model of total cavopulmonary connection to test the response to axial flow pump (Thoratec HeartMate II; Thoratec Corporation (Pleasanton, Calif)) support of total cavopulmonary connection, which might be suitable to treat patients with failing Fontan circulation. METHODS: Eight sheep (42-48 kg) were studied. After pilot studies in 3 animals, 5 underwent both pump-supported and nonsupported total cavopulmonary connection in alternating sequence for up to 2 hours. This was achieved with a 12-mm polytetrafluoroethylene graft from the (distally ligated) superior vena cava to the main pulmonary artery and a cannula placed in the inferior vena cava with an attached 16-mm Dacron graft to the main pulmonary artery. Pressures (arterial, inferior vena cava, left atrium, and pulmonary artery) and flows (ascending aorta and inferior vena cava) were recorded over 1 hour both with unsupported total cavopulmonary connection and after placing an axial flow pump (Thoratec HeartMate II) between the inferior vena caval inflow cannula and the main pulmonary artery. RESULTS: Under nonsupported total cavopulmonary connection circulation, inferior vena caval and aortic blood flow decreased by nearly 50%. Inferior vena caval pressure nearly doubled, whereas arterial pressure decreased by one third. Pulmonary artery pressure became nonpulsatile; however, mean pulmonary artery pressure and left atrial pressure did not change significantly. With pump-supported Fontan circulation, cardiac output, inferior vena caval flow, and arterial pressure returned to baseline. Inferior vena caval pressure decreased to below baseline levels. Mean pulmonary artery pressure and left atrial pressure again remained unchanged. CONCLUSIONS: Axial flow pump support from the inferior vena cava to the pulmonary artery can prevent the substantial decrease of aortic flow and pressure associated with total cavopulmonary connection and can reverse its poor hemodynamics. This is a simple model that can be used to further evaluate the potential of mechanical support as a treatment option in failing Fontan circulation.     

Cardiac and Great Vessel Involvement in “Behcet's Disease”

Abstract Behcet's disease is a multisystem disorder and classified as “vasculitic syndrome with a wide variety of clinical manifestations.” Cardiac involvement is very rare but can occur with different presentations including: pericarditis, cardiomyopathy, endocarditis, endomyocardial fibrosis, intracavitary thrombosis, and coronary artery disease. Great vessel involvement is more common. Recurrent Phlebitis, commonly involving large vessels (superior vena cava, inferior vena cava, hepatic veins) and cerebral veins are the sole presentation in this regard. Arterial involvement is expressed by aneurysm or pseudoaneurysmal formation. Due to the wide variety of cardiovascular manifestations and the resulting high mortality, cardiac surgeons should be familiar with this disease. In this paper we review the articles and introduce our four cases presenting with aneurysm of ascending aorta with free aortic insufficiency, aneurysm of descending aorta, pulmonary artery aneurysm, and pseudoaneurysm of aortic arch.     

Distribution of hepatic venous blood in the total cavo-pulmonary connection: an in vitro study

Objectives: The objective of this project was to quantify the effects of geometry on the distribution of hepatic blood to the lungs in patients with a total cavo-pulmonary connection. The basis for this work is the supposition that hepatic blood is necessary for proper lung function. Methods: Plastic models of these connections were made with varying degrees of offset between the inferior and superior vena cava and attached to an in vitro flow loop. Dye was injected into the inferior vena cava and its concentration quantified in each pulmonary artery. These data were converted to percentage concentration and distribution of hepatic blood to each lung. Results: With no offset between the vena cava, hepatic blood distribution and concentration to each lung was similar to normal. For an offset of one or more diameters, hepatic blood tended to flow preferentially towards the nearest pulmonary artery with the opposite pulmonary artery exhibiting a deficit (<10% of normal). Conclusions: Distribution of hepatic blood to each lung was found to be a function of vena cava offset and pulmonary artery flow split. Under normal conditions, 60% of blood towards the right pulmonary artery, the hepatic blood distribution to both lungs could be maintained above 50% of normal if the inferior vena cava was offset towards the left pulmonary artery. Offsetting the inferior vena cava towards the right pulmonary artery jeopardized the delivery of hepatic blood to one lung.     

Unusual course of a pulmonary artery catheter through a persistent superior vena cava]

We report the unusual course of a pulmonary artery catheter through a persistent left-sided superior vena cava. After left subclavian vein cannulation and downward left-sided paramediastinum course, the Swan-Ganz catheter enters the right pulmonary artery. Haemodynamic monitoring was consistent with the diagnosis of septic shock developed by this 56-year-old woman, after cephalic duodenopancreatectomy. Persistent left-sided superior vena cava occurs in 0.5% of the population and 5-10% of patients with congenital heart diseases. It drains into the right atrium through the coronary sinus in 92% of cases and is associated with an absent right superior vena cava in 20% of cases. The left-sided superior vena cava persists when the caudal part of the left anterior cardinal vein does not degenerate. A persistent left superior vena cava may be medically relevant during implantation of pacemaker leads or radiofrequency ablation, during cardiac surgery for placement of a retrograde coronary sinus cardioplegia catheter and during transjugular intrahepatic portosystemic shunt placement.     

Surgical treatment of circumflex coronary aneurysm with fistulous connection to coronary sinus associated with persistent left superior vena cava

Circumflex coronary arteriovenous fistula associated with aneurysmal dilatation and draining into coronary sinus (CS) is rare. A 57-year-old female presented with progressive dyspnea and was found to have a tortuous multiloculated aneurysm of the circumflex coronary artery terminating into the CS associated with a persistent left superior vena cava. The operative repair and management of coronary arteriovenous fistula are the subject of this case report and review.     

Transcaval invasion of right atrium by thymoma: resection via transient cava-pulmonary shunt

Transcaval extension of the thymoma to the right atrium has very rarely been reported, and cardiopulmonary bypass is recommended for successful resection. An invasive thymoma with intravascular invasion of the superior vena cava, and the left innominate vein extending into the right atrium was presented. Intra-atrial extension was resected through a transient external shunt from the inferior vena cava to the main pulmonary artery. We discussed the feasibility of this surgical technique and possible advantages of cardiopulmonary bypass avoidance.     

Left inferior vena cava with renal cell carcinoma

We presented a case of inferior vena cava on the left side diagnosed by abdominal CT scan during the study of a renal cell carcinoma in the right side. This anomaly is very rare but it may suppose a more difficult approach to the nephrectomy.