Case of the Month- One Stage Correction of Ascending Aortic Aneurysm and Coarctation of Aorta in an adult.

A 38 year old gentleman presented with the complaints of chest pain and dyspnea on exertion (NYHA II) for 3 months. He was evaluated by his GP and found to be hypertensive. Cardiomegaly was observed on chest X Ray and he was sent for further evaluation.

Echocardigrphy revealed a bicuspid aortic valve with severe incompetence with dilated ascending aorta. A CT angiogram was performed which revealed a 8.5 cm aneurysm of the ascending aorta involving the aortic sinuses. The arch was hypoplastic with evidence of tight coarctation of aorta just after the origin of the left subclavian artery.( Image Below)

Post operative photo.

The patient underwent a single stage correction of the defect via a median sternotomy. Innominate artery was used formarterial cannulation. A 8mm dacron graft was used to cannulate this artery. Bicaval venous cannulation was used for venous return. Custodial cardioplegia was used for myocardal protection. A Bentalls procedure was performed with a size 23 valve conduit. With the heart arrested it was retracted to the right using the Starfish retractor. The descending aorta was dissected out posterior to the pericardium. A size 20 dacron graft was sutured to it end to side. This graft was then brought over the diaphragm, IVC and the right atrium to the right side of the heart. It was then connected to the aortic valved conduit in an end to side fashion. An extra anatomic bypass of the coarctation was therefore created.(Images below). There was no gradient of pressures between the upper and lower limbs post op.

Post operative CT.

Comment- Coarctation of the aorta may occassionaly be diagnosed in adulthood. It sis often associated with disease of the aortic root and valve. Traditionalyl, a staged operation requiring a median sternotomy and thoracotomy were performed to correct this defect. In the recent years a single stage approach as described above is recommended. The morbidity is less and the fragile area of the coarctation is not touched at all.  

Case of the Month- Pulmonary Embolism Masquerading As Acute Decompensation In A Patient With Severe Aortic Stenosis.

A 58-year-old gentleman with known aortic stenosis, presented with acute onset of New York Heart Association Class IV dyspnoea and bi-ventricular failure. His transthoracic echo revealed severe calcific aortic stenosis, severe pulmonary arterial hypertension with severe bi-ventricular dysfunction. There was also a large mobile mass in the right atrium projecting into right ventricle the presence of which was confirmed on trans-esophageal echocardiography (Fig.1).

The patient was taken up for urgent surgery. Per-operatively, a linear clot, measuring eighteen centimeters was found in the right atrium (Fig.2). It was extending through the tricuspid valve into the right ventricle. The clot was removed. A small patent foramen ovale was present which was closed directly. Aortotomy was then performed and aortic valve replacement was done with a mechanical valve.

After the patient was shifted to ICU, a peripheral venous Doppler revealed bilateral deep vein thrombosis extending into femoral veins. An inferior vena caval filter was deployed.

The patient was discharged on oral anticoagulation.

This case demonstrates that in patients with pre-existing heart disease who present with sudden de-compensation, acute pulmonary embolism should also be considered and investigated for.

 

Figure1.

 

Figure 2.

Case of the Month - Solitary Fibrous Tumor of the Pleura(SFTP)

 

Case History-

A 70 year old lady presented with worsening dyspnea over last 3 months. For the last 2 weeks she was unable to perform her daily chores.

She was evaluated by her GP and a chest x-ray and CT Chest were done (image below).

 

 

The chest X ray showed opacification of the left hemithorax with shifting of the mediastinal structures to the right.
The CT chest showed a large mass (17cm X 8cm X 7cm) occupying the anterior portion of the left hemithorax. It was of homogenous density with areas of calcification. The upper lobe of the lung was completely obliterated. The mass was abutting the left hilar vascualr structures without any evidence of invasion. The mediastinal structures were shifted to the right due to the mass effect.
A FNAC of the mass was performed at another hospital was neagative for malignant cells.

The patient was taken up for surgery after necessary work up. In view of the close proximity to the hilar structures, I decided to open the chest with the clamshell approach(B/L thoracotomy- figure below). The hilar structures (left pulmonary artery and vein) were controlled intrapericardially with vessel loops. The tumor was then dissected out. It was non adherent to the vascular structures and could be delivered out of the chest cavity after lysing flimsy adhesions to the chest wall and the lung. There was a vascular pedicle from the lingula region which was ligated and divided. The left uppel lobe of the lung which was compressed by the tumor was spared and expanded fully once ventilation was restored.
The tumor weighed 1.75kg (figure below).
The gross findings were suggestive of a solitary fibrous tumor of the pleura (SFTP) and the histopathology report was confirmatory. The tumor showed uniform spindle cells with connective tissue in between. There was no evidence of malignancy.

Clamshell Incision



Comment- Solitary fibrous tumor of the pleura is a mesenchymal tumor that has been increasingly recognised over the past few years. Majority of these tumors have a benign course cured by simple excision. About 12% have malignant course and may recur. The case above is a classical presentation of this tumor. The main differential diagnosis include - pleural mesothelioma, neurogenic sarcoma, synovial sarcoma, hemangiopericytoma, fibrosarcoma and malignant fibrous histiocytoma. Immunohistochemical staining can be used to confirm the diagnosis.

Case of the Month- Post MI Posterior LV Aneurysm- Endoventricular patch Repair.

Case of the Month- Post MI Posterior LV Aneurysm- Endoventricular patch Repair

 

A sixty year old diabetic male presented to us with a three week history of chest discomfort accompanied by sweating, restlessness and uneasiness.

He was evaluated elsewhere for coronary artery disease but the echo was found to be unremarkable. His chest X- ray revealed left pleural effusion, and he was empirically started on ATT. The patient did not improve and came to us for review/second opinion.

We conducted the following tests:

ECG- t wave inverted infr. Leads                                                                                              

CT Chest Contrast- pericardial collection, postr. Aneurysm.

 CXR- lt pleural effusion

 

 

Echo-

 Posterior LV aneurysm with pericardial effusion.

 Coronary Angio-                Occluded RCA.                      

 Proximal LAD 70-80%.

 

In view of the echo findings and the symptoms, a diagnosis of post MI, aneurysm with contained rupture of the posterior LV wall was made.

A coronary angiography was performed, which revealed significant lesions of LAD and complete occlusion of RCA.

The patient was taken up for emergency surgery. Per-operatively there were dense adhesions in the pericardium. A large 6cmX3cm true aneurysm of the posterior LV wall was found. The patient underwent CABGX2 with LIMA to LAD and SVG to PDA. The aneurysm was excised and defect in the left ventricle repaired with a Dacron patch. (Endoventricular patch). The patients lt pleural fluid was sent for examination and was negative for tuberculosis.

The patient made an uneventful recovery. The post- operative echo shows-  normal lv function, minimal pericardial effusion (post surgery).

Comment-

LV aneurysms complicate 10% of MI’s. Most of them have a chronic course and a good prognosis on medical management. Surgery is indicated when symptoms appear. The usual symptoms are, - angina, Dyspnoea and ventricular arrhythmias causing syncope. On echo an EDV of more than 120ml/m2 is also a relative indication. Rupture is a life threatening condition requiring urgent surgery.

Indications for Surgery.

Documented expansion/large size Angina Congestive heart failure Arrhythmia Rupture Pseudoaneurysm Congenital aneurysm Embolism Documented expansion/large size

 

Surgery consists of CABG (if there is significant CAD on angiography) along with excision of aneurysm and repair of defect in LV with a Dacron patch. The operative mortality is 4-7% with a 80% five year survival.

 

 

 

Punjab's First freestyle Porcine Valve Implant.

Last year we operated on a fifteen year old girl with severe aortic stenosis. She had the rare birth defect of unicuspid aortic valve. Her heart function had come down to 25% and she could barely walk. Her annulus size and ascending aorta were very small. Implanting a regular mechanical valve would have been futile as she would have high residual gradients, also she would have to be on life long anticoagulation.
I therefore implanted a freestyle valve as a complete root replacement technique. This valve is a commercially available pig valve. In a sense it is like transplanting a pig valve in the patient. The advantages are that there are minimal gradients and the heart function recovers promptly. Also the girl need not be on anticoagulation and can have a normal family life. Needless to say the procedure is technically very demanding and time consuming.
At one year follow up the girl is healthy with normal heart function.
The company reps tell me that this is the first time such a valve has been implanted in Punjab!!!



Freestyle Porcine(pig) aortic valve.

Total Arterial Coronary Bypass- Bilateral Mammary Arteries.

Case of the Month- Annulo aortic ectasia- Bentalls Operation.

A 66 year old male referred for evaluation of palpitations and dyspnoea on exertion(class III). ECG was suggestive of left ventricular hypertrophy. Transthoracic echocardiography revealed a tricuspid aortic valve with moderately severe aortic regurgitation. The aortic annulus was dilated (30 mm). There was dilation of the ascending aorta (6.7cm). A  CT aortogram was done (see images below), which showed dilatation of the aortic annulus and the aortic sinuses with aorta tapering to a near normal diameter just before the arch vessels.The images were typical of annulo aortic ectasia. the coronary arteries were normal on angiogram.

Classical pear shaped appearance of aortic root.

The patient underwent a aortic root replacement (modified Bentalls operation)using a size 25 valved conduit.


Comment-Annulo aortic ectasia is a condition in which there is dilatation of the aortic annulus and the aortic sinuses. this leads to stretch on the aortic valve leaflets resulting in variable degrees of aortic regurgitation. This condition is found in patients with Marfans syndrome but aging and hypertension are also the causes. The basic defect is weakness of the medial layer of aorta resulting in dilation which progresses with time. As the size of the aorta increases, the chance of dissection, rupture also increase. The treatment of this condition is surgery. There are well defined guidelines for aortic size when intervention is indicated. If the aortic valve can be preserved, a valve sparing (David I) operation can be performed. In others the aortic valve and the aorta are replaced using a composite graft (Bentalls operation) with reimplantation of the coronary arteries.
Other conditions in which Bentalls procedure is performed are type A aortic dissection with irreparable aortic valve, symptomatic bicuspid aoric valve disease with ascending aortic dilatation. The current aortic size guidelines above which ascending aorta should be replaced  are:

Bicuspid aortic valve- 4.5 cm.
Marfans Syndrome-  5.0 cm
Others------------- 5.5 cm

Composite graft and valve conduit

Modified Bentalls procedure