Left atrial myxoma is the most common variant of cardiac tumour, rarely presenting with systemic embolization involving 3 limbs and intra cranial bleed simultaneously. Our case aims to discuss appropriate management strategy in such cases. This case presents a 34 year old lady with acute limb ischemia both lower limb and right upper limb with intracranial bleed with no signs of congestive heart failure diagnosed with LA Myxoma on preoperative routine 2d ECHO cardiography. She underwent embolectomy of right brachial artery and bilateral femoral artery, common iliac artery and abdominal aorta with excision of LA Myxoma planned for 4 weeks later. In patients presenting with acute limb ischemia and intracranial bleed, the definitive surgery of LA myxoma excision should be postponed for 4 weeks to prevent risk of systemic heparinisation causing worsening of neurological functions while acute limb ischemia should be addressed immediately.

The arterial switch operation (ASO) is the procedure of choice for the management of d-transposition of the great arteries (TGA). However, the surgical management of infants older than 6 weeks with TGA and intact ventricular septum (IVS) remains contentious. We report a case of late presenting TGA, IVS with Systolic anterior motion(SAM) of mitral valve with preserved left ventricle(LV) and its management.

DISCUSSION – Left brachiocephalic vein is 6 cm long; it begins behind the sternal end of the clavicle, anterior to the cervical pleura by union of the left internal jugular vein and the left subclavian vein. It descends obliquely to the right, behind the upper half of the manubrium sterni, up to the sternal end of the first right costal cartilage, uniting here with right brachiocephalic vein to form SVC. The aortic arch is inferior to this vein. The left retro-aortic bracheocephalic vein is rare entity which was first described by Kerschner.[(1)](#ref-0001) Incidence of left retro-aortic bracheocephalic vein is noted between 0.2 to 1%.[(2)](#ref-0002) Aorto-pulmonary window is a rare cardiac condition, first described by Eliotson in 1830[(3)](#ref-0003) with an incidence of 0.2% - 0.3% of all congenital cardiac lesions.[(4,5)](#ref-0004) Three standard approaches for surgical closure of aorto-pulmonary window have been described in literature include: trans-aortic approach, trans-window approach, trans-pulmonary approach. Trans-window approach is also known as sandwich repair of aorto-pulmonary window.[(6)](#ref-0006) The left retro-aortic brachiocephalic vein with tetralogy of fallot and coarctation of aorta are noted. The left retro-aortic brachiocephalic vein with aorto-pulmonary window is very rare case and has not been reported previously. In our case the patient was admitted for AP window closure after complete pre-operative evaluation. Cardiac computed tomography reported 2.2cm aorto-pulmonary window with the left retro-aortic brachiocephalic vein. After obtaining parental consent for surgery, median sternotomy was performed. Thymus was excised and pericardium was opened longitudinally. By careful dissection, the left brachiocephalic vein was identified behind the distal ascending aorta adjacent to the AP window. Aorta was dissected off the brachiocephalic vein meticulously. Due to left retro-aortic brachiocephalic vein, aortic cannulation had to be done more caudally to avoid obstruction of vein. Aorta was cannulated and clamped without injury to the brachiocephalic vein. Attention was paid to avoid injury of the brachiocephalic vein during encircling the SVC for snaring. Aorto-pulmonary window was closed with PTFE patch through trans-window approach under mild hypothermia.[(6)](#ref-0006) Clinical implication of retro-aortic brachiocephalic vein is very important. More caudal cannulation of SVC is required in left brachiocephalic vein; but in AP window setting, this is more difficult as work space for closure of the defect is also required. Injury may occur to the vein during clamping of aorta. Left retro-aortic brachiocephalic vein may cause technical difficulty during central venous line placement through left arm approach. CONCLUSION- Being the rarest combination of left retro-aortic brachiocephalic vein and aorto-pulmonary window, certain things have to be taken care of like clamping of aorta, SVC cannulation, central vein catheter insertion, snaring of SVC. Pre-operative CT scan is also important to avoid intra-operative surprises.

Pseudoaneurysm of the right ventricular outflow tract(RVOT) is an uncommon yet catastrophic complication after intracardiac repair of Tetralogy of Fallot(TOF). We describe a patient diagnosed with RVOT pseudoaneurysm in the immediate postoperative period after complete repair for TOF with single pulmonary artery. The pseudoaneurysm was repaired successfully. This case is reported to emphasise the importance of a high degree of suspicion of this rare entity in these patients for its early diagnosis and management.

Background: Pulmonary Hypertension in mitral valvular heart disease leads to various adverse outcome following surgical treatment of this condition. In majority of the patients this Pulmonary Hypertension is reversible following surgery. The objective of this study is to assess the outcome of changes in Severe Pulmonary Hypertension after Mitral Valve Replacement during the follow up with postop Echocardiogram. Patients and methods: In all our 265 patients who underwent Mitral Valve Replacement Surgery (MVR) for Mitral valvular heart disease with pulmonary hypertension, females 111/195(56.9%) outnumbered the males in this study. 195 among 265 cases had severe PHT. MVR was done with cardiopulmonary bypass using St Jude’s medical valve. Results: Despite the high operative mortality in most series of MVR in patients with severe PHT, a striking improvement in survival was noted in the study with 5.3% mortality rate.A sudden drop of PHT is not observed in our study. The fall was found to be gradual and significant over follow up and the maximum reduction was at 1-3 months post operatively. Conclusion: It is concluded that MVR reduces PHT in a gradual way and the mortality rate is also minimum in severe PHT patients. MVR thereby is an effective invasive procedure for the management of patients with severe mitral valve disease and PHT. Keywords: Severe Pulmonary Hypertension, mitral valvular disease, mitral valve replacement, Pulmonary artery pressure, Rheumatic Heart Disease

Left ventricular free wall rupture (LVFWR) can occur after myocardial infarction. Sometimes LVFWR can be contained by a pericardium leading to the formation of the pseudoaneurysm. In view of the high tendency to expand and rupture an urgent surgery is indicated. We experienced a rare case of giant left ventricular apical pseudoaneurysm which was successfully managed with surgical intervention.

Tetralogy Of Fallot (TOF) with absent pulmonary valve (APV) and unilateral absence of pulmonary artery (UAPA) is an extremely rare congenital cardiac anomaly. “Complete” unilateral absence implies absence of both intrapericardial and hilar segments. Co- existence of both free pulmonary regurgitation (PR) and decrease in the cross sectional area for right ventricular outflow tract (RVOT) in this substrate, are detrimental for right ventricular (RV) function. Early intervention to preserve RV function is imperative. Durable and competent valve in RVOT can be extremely helpful in improving the overall prognosis. We present a case of a two year old child, TOF with APV, absent left pulmonary artery (ALPA) who underwent RVOT reconstruction, with a pulmonary valve (PV) made from polytetrafluorethylene (PTFE) membrane. To our knowledge construction of PV using Graham Nunn technique, has not been reported with this morphology.