Congenital unilateral pulmonary vein atresia: A rare disease with successful outcome in a child.

Unilateral pulmonary vein atresia is a rare congenital anomaly that may occur in association with other congenital heart diseases. We report a rare case of left-sided pulmonary vein atresia in a 2-year-old child who presented with recurrent hemoptysis and was managed surgically with left pneumonectomy.

How We Do It: Whole Lung Lavage.

Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disorder characterized by the accumulation of surfactant in the alveolar spaces resulting in hypoxemic respiratory failure. Whole lung lavage (WLL), the preferred treatment for PAP, physically removes the lipoproteinaceous material from the alveolar spaces. Since its initial description in 1963, the WLL procedure has undergone various modifications. However, the procedure has not been standardized yet. After securing a double lumen endotracheal tube, we perform WLL under general anesthesia. One lung is ventilated, while the other is lavaged using one-liter aliquots of pre-warmed saline. We use gravity-assisted drainage of the lavaged lung after each cycle till the milky white and opaque fluid becomes clear (usually 15-20 cycles). Herein, we describe the step-by-step procedure, precautions, and monitoring of WLL. We also provide videos demonstrating one-lung ventilation and bronchoscopic confirmation of lung isolation.

A single coronary artery with left circumflex artery crossing right ventricular outflow tract in tetralogy of Fallot with absent left pulmonary artery.

Tetralogy of Fallot (TOF) with unilateral absence of pulmonary artery and the anomalous coronary artery is a rare combination. Detailed preoperative evaluation of coronary artery anatomy is must to prevent injury to the major vessels crossing right ventricular outflow tract. We report a rare association of single coronary artery with left circumflex artery crossing right ventricular outflow tract close to the pulmonary annulus in tetralogy of Fallot with absent left pulmonary artery in 11-year-old girl. Though there is a great diversity of coronary anomalies in tetralogy of Fallot, the prepulmonic course of left circumflex artery crossing the right ventricular outflow tract (RVOT) close to the pulmonary annulus has rarely been described in the literature. The patient underwent successful primary single lung intracardiac repair. Right ventricular outflow tract obstruction was treated by handmade valved pericardial autologous conduit and release of the tethering of hypoplastic native unicuspid pulmonary valve leaflet maintaining its integrity.

Surgical Valve Repair of Native Pulmonary Valve Endocarditis Using the Ozaki Technique.

Surgery for infective endocarditis in the pulmonary position is an effective method of treatment despite a very uncommon pathology and few operations being performed. We present an adult male patient with right-sided infective endocarditis where the pulmonary valve cusps were almost completely damaged. The pulmonary valve was successfully reconstructed using glutaraldehyde-treated autologous pericardium by the Ozaki technique. The Ozaki repair was originally described for aortic valve repair in various aortic valve diseases.

ST Segment-Elevation Myocardial Ischemia After Arterial Switch Operation due to Pericardial Drain Placement: A Case Report.

Myocardial ischemia postarterial switch operation has been reported extensively in the literature and has been almost exclusively ascribed to issues related to coronary artery translocation. Here, we report a case of a 5-week-old child with D-transposition of the great arteries who underwent an arterial switch operation and developed myocardial ischemia after pericardial drain placement, as evidenced by significant ST segment elevation and abnormal regional cardiac wall motion. The ST segment and cardiac wall motion improved once the drain was withdrawn from the pericardium and placed in the retrosternal space. Few reports in the literature describe this complication, particularly in pediatric patients.

A case of traumatic thoracic aorta rupture - A life threatening emergency.

Traumatic aortic transection is a life threatening emergency where there is a near-complete tear through all the layers of the aorta due to trauma. This condition is most often lethal and requires immediate medical attention. Symptoms of an aortic rupture may include severe chest pain, back pain, abdominal pain and signs of external chest injury. Treatment should be prompt in hemodynamically unstable patient in the form of endovascular or open surgical technique. We present a twenty nine year old male with aortic transection following motor vehicle accident where an interposition tube graft was placed after trimming the lacerated segments of the aorta under cardiopulmonary bypass. The patient is doing well with two years of follow up at our institution.

Tricuspid stenosis: A rare and potential complication of ventricular septal occluder device.

Asymmetrical septal occluder device (ASOD) has made percutaneous closure of ventricular septal defect an easy and effective management option. Although there are reports of aortic and tricuspid valvular regurgitation after deployment of ASOD, only few cases of tricuspid stenosis (TS) has been reported so far in the literature. We report a case of malaligned ASOD that occurred after successful device closure resulting in TS along with mild tricuspid and aortic regurgitation requiring surgical retrieval. Transesophageal echocardiography played crucial role in detecting the cause of tricuspid valve dysfunction besides providing continuous monitoring during the procedure. We intend to emphasize the need of echocardiographic evaluation of the tricuspid valvular apparatus and aortic valve during and after the device deployment even after the successful device closure to prevent this rare complication.

Tricuspid valve straddling: An uncommon cause of left ventricular outflow tract obstruction in transposition of great artery with ventricular septal defect.

Transposition of great arteries (TGA) can be associated with left ventricle outflow tract (LVOT) obstruction. In the presence of ventricular septal defect (VSD), septal leaflet of tricuspid valve may prolapse through perimembranous VSD or rarely tricuspid valve tissue may override to produce LVOT obstruction. Occasionally, this may be mistaken for vegetation due to associated pulmonary valve endocarditis. We report a case of d-TGA with presumptive pulmonary valve endocarditis and LVOT obstruction that was found to be due to tricuspid valve straddling on transesophageal echocardiography, resulting in change in the surgical plan and thus avoiding catastrophe.

Wilm's tumor with intracardiac extension causing dynamic tricuspid valve obstruction: An anesthetic challenge.

Wilms' tumor (WT) is the most common pediatric renal tumor that often spreads to inferior vena cava and sometimes up to right atrium (RA). We describe successful management of 3-year-old child diagnosed with WT having extension up to RA. He was operated under cardiopulmonary bypass and extubated on postoperative day 2 and discharged. Perioperative anesthesia concerns were shock from dynamic tricuspid valve obstruction, intraoperative massive blood loss, and a higher risk of pulmonary thromboembolism during tumor manipulation.

Silicone tracheobronchial stent: A rare cause for bronchoesophageal fistula and distortion of airway anatomy.

Silicone tracheobronchial stents are being increasingly used in a large number of patients for the treatment of tracheal stenosis. One very rare complication due to tracheobronchial stenting is bronchoesophageal fistula (BEF), which has been associated with the use of metallic stents. We report intraoperative management of a patient undergoing repair of a BEF, following previous insertion of a silicone Y-stent that is soft in texture and has not been implicated for this complication till date. In addition, misalignment of this silicone tracheobronchial Y-stent resulted in a tracheal mucosal bulge proximal to the stent that vanished after its removal.