Partial anomalous pulmonary venous drainage of the left lung into the innominate vein (author's transl)]

From 5 cases of partial anomalous pulmonary venous drainage of the left lung into the innominate vein, it has been thought of interest to situate this type of venous anomaly among the partial anomalous pulmonary venous drainage classification. It is revealed in 2 different ways. In adults, this malformation sets the problem of the diagnosis of an abnormal chest Xray, without symptom. In children, this vascular abnormality is discovered during the exploration of a congenital cardiopathy which causes the main symptoms. Embryological data are reviewed and statistics of frequency which lead to show that this type of partial anomalous venous drainage is often misunderstood, particularly with regard to anomalous venous drainage of the right lung.     

A case of anomalous pulmonary venous drainage from the entire left lung associated with complete heart block.

A 22-year-old female patient presenting complete atrioventricular block and giant P waves in electrocardiogram had anomalous pulmonary venous drainage from the entire left lung. There was normal drainage from the right lung and no associated atrial septal defect or other intracardiac abnormalities. After a permanent pacemaker was implanted, she manifested signs and symptoms of heart failure. Although the anomalous pulmonary vein was anastomosed to the left atrium, intractable heart failure continued. She died six months later after surgical intervention. Postmortem examination revealed diffuse interstitial fibrosis throughout the myocardium.     

Association of cor triatriatum and obstruction of abnormal left pulmonary venous drainage. Apropos of a case

A case of subtotal cor triatriatum associated with a partial anomalous pulmonary venous drainage of the left lung, in a 30 months old boy is reported. Diagnosis was suggested by the presence of pulmonary venous obstruction and pulmonary hypertension. Doppler, echocardiogram diagnosed the cor triatriatum and the degree of the pulmonary hypertension. The cine-angiography demonstrated the anomalous, obstructive, left pulmonary venous drainage. The pulmonary hypertension has mandatorised an early surgical cure, the result was satisfactory at 16 months.     

Masked abnormal drainage of the inferior vena cava into the left atrium

A 6 year old boy with a large atrial septal defect, partial anomalous pulmonary venous drainage and unrecognized anomalous insertion of the inferior vena cava into the left atrium had cyanosis after closure of the atrial defect. Repeat study revealed direct drainage of the inferior vena cava into the left atrium with moderate arterial oxygen desaturation. At repeat operation an unusual positioning of the inferior vena cava was seen. After reopening of the atrial defect, the pulmonary venous and systemic venous drainage anomalies were identified. A Dacron patch was inserted so as to divert flow to the proper atrium. Repeat catheterization 3 months after operation revealed a normal heart with no obstruction; arterial oxygen saturation was normal. The child has continued to do well 3 years after operation.     

Transcatheter therapy in partially abnormal pulmonary venous return with additional drainage to the left atrium

Background

A persistent anastomosis between the pulmonary veins that connect with the left atrium and the systemic vein that drains into the right atrium has occasionally been reported. We report characteristics and transcatheter therapy in partially abnormal pulmonary venous return with additional drainage to the left atrium.

Methods

We retrospectively studied such patients in 5 institutions.

Results

Ten patients (6 girls) presented at a median age of 8 (0.1 to 54) years with 2 anatomic types: 8 vertical vein types with drainage of the left upper lobe to the innominate vein via a large vertical vein (left superior cardinal vein) and to the left atrium via the left upper pulmonary vein; and 2 scimitar vein (SV) types with drainage of the right middle and lower pulmonary veins into the inferior vena cava and to the left atrium via an anomalous connecting vein. Associated malformations were aortic coarctation (n = 2) and secundum atrial septal defects (n = 3). Two patients of the vertical vein type were operated. Transcatheter occlusion of the abnormal pulmonary venous return was performed in 7 cases, associated with occlusion of systemic arterial supply (n = 2), secundum atrial septal closure (n = 2), left upper pulmonary vein stenosis stenting (n = 1), and coarctation stenting (n = 1). Including previously published cases, 18 patients (13 vertical veins and 5 scimitar veins) underwent transcatheter repair. Patients over 40 years of age tend to be symptomatic at presentation (p = 0.056).

Conclusion

In partially abnormal pulmonary venous return with dual drainage, transcatheter therapy can be offered in the majority of patients.     

Catheter intervention for abnormal pulmonary venous drainage

Transcatheter interventions for congenital heart anomalies are constantly improving. Although correction of anomalous pulmonary venous connection is routinely achieved through surgery, there are rare instances where the abnormal pulmonary vein has dual connections to both left atrium and the major systemic veins. Under these circumstances catheter based treatment might become a feasible option. We report a case of dual supply vertical vein connected to left upper pulmonary vein and innominate vein which was successfully obstructed by an occluder device leading to improvements in patient’s condition.     

Cyanosis in patients with atrial septal defect due to systemic venous drainage into the left atrium

Cyanosis and clubbing of the digits in patients with atrial septal defect usually are indicative of reversal of the interatrial shunt consequent to reduced right ventricular compliance and severe pulmonary hypertension. The condition may be considered inoperable. The cases reported herein emphasize the occurrence of cyanosis in patients with interatrial communication and continued significant left to right shunting, as a consequence of systemic venoarterial shunting into the left atrium. Carefully planned cardiac catherization is indicated to detect the less well recognized mechanisms of cyanosis so that such patients are not denied the benefits of surgical closure of the septal defect.     

Angioplasty of the common trunk of the left coronary artery with venous patch

Surgical angioplasty of the left main coronary artery for severe iatrogenic stenosis has been done on a 40 year old female patient operated upon five months before for mitral and aortic valve prosthetic replacement. Postoperative angiocardiographic study showed patent left main trunk and the patient is angina-free six months post-operatively. Operative indication and surgical technique are discussed as an alternative to aorto-coronary by-pass surgery for left main proximal stenosis without peripheral lesions. It does not result from literature that this surgical technique has ever been employed in patients previously operated with open heart surgery.