Category: Case Study

A 77-year-old American national was admitted with history of palpitation, chest pain and shortness of breath. He was very fragile and weighed only 48 kg. He was diagnosed to have severe rheumatic mitral regurgitation, severe tricuspid regurgitation, atrial fibrillation, and left ventricular failure. He had an attempted mitral clip procedure in another hospital, which failed. He was a known case of stage 4 chronic renal failure, sickle cell anemia, thrombocytopenia, diabetes mellitus and visual defects as tubular vision. The patient had multiple history of sickle cell crisis. Two years back he was admitted with septicemia, comatosed, ventilated and had tracheostomy in a hospital in the USA. This case was discussed in our heart failure clinic and accepted for the high risk procedure.

Surgical technique

All precautions were taken to deal with problems of sickle cell disease on cardiopulmonary bypass. The patient had exchanged transfusion before instituting cardiopulmonary bypass. He was hydrated well and 2.5 L of blood was removed at the beginning of CPB to a separate cardiotomy reservoir and discarded. CPB was started with blood prime to replace the lost volume and kept on normo-thermic. Operating room temperature was kept at 25 degrees C. Ultra-filtration was also used. The mitral valve was replaced with 27 mm bioprosthetic valve and tricuspid valve was repaired with 30 mm MC3 annuloplasty ring. Transatrial closure of the left atrial appendage was done using two-layer suture. The patient came off bypass with stable hemodynamics on minimal inotropic support. In the postop period, all precautions were taken to avoid sickle cell crisis. He had a smooth outcome and was discharged on 15th post-op day. He is being followed up in our clinic and is doing well.

Discussion

Patient with pre-op high morbidity underwent a very high risk procedure. This case was initially managed in another cardiac institute and decided not to subject him for open heart surgery due to his comorbidities. Hence mitral clip procedure was attempted, but failed. Following this, he had multiple admissions with pulmonary edema. The family support and the patient’s motivation was great and encouraged us to take him up for surgery. This helped to achieve a good result.

Acute rupture of anterior papillary muscle of mitral valve – emergency institution of cardiopulmonary bypass in the ICU

History

A 51-year-old male patient, known case of hypertension, hyperlipidemia, and diabetes mellitus type II (not on regular medication), presented to another hospital after collapse at work. He was having chest pain for previous 3 days, had not sought any help, till he collapsed. In the Emergency room he was having acute shortness of breath and hypoxia. He was intubated in the Emergency Room. He had a massive pulmonary edema. His investigations showed highly elevated cardiac markers (Tro-ponin T > 3000 pg/mL and Pro BNP > 3000 pg/mL). He was taken up for coronary angiogram, which showed triple vessel disease with acute thrombosis of RCA. Balloon Angioplasty was done to RCA. In view of his cardiogenic shock and severe hypo-tension, despite heavy inotropic support (Nor Adrenaline and Adrenaline), he was put on IABP. As his condition did not improve, cardiac surgery team at Burjeel was consulted and immediate transfer was advised. On arrival, he was in renal shut-down and was developing hepatic dysfunction. Acidosis persisted and despite corrective measures and adjusting ventilator parameters, his condition deteriorated. He was given multiple doses of Phe-nylephrine injection and a Methyl-ene Blue infusion was started. His heart rate remained above 140/min. Transthoracic echocardiogram showed fair right and left ventricular contractility. Despite very poor prognosis for any intervention, the relatives granted permission and requested to go ahead with any procedures. A bedside VA ECMO was instituted with centrifugal pump through femoral cannulation. He developed VT & VF, during the procedure, needing defibrillation. Gradually he got stabilized with ECMO, the heavy dose of inotropic support could be reduced with mean pressure of 60-65 mmHg. His heart rate came down to 70-80/min in normal sinus rhythm. TEE was done. Severe Mitral Regurgitation was observed with anterior papillary muscle rupture. His condition was explained to the relatives. In view of the refractory cardiogenic shock, unstable hemodynamics, despite IABP and ECMO, it was decided to proceed for Emergency CABG + MVR as a desperate life-saving measure.

Surgical Procedure

Intra Operatively ECMO was converted to Cardio Pulmonary Bypass. CABG x 3 and Mitral Valve was replaced with 25mm size CE bioprosthetic valve. As anticipated, he had difficulty in coming off cardiopulmonary bypass; hence, VA ECMO was reinstituted through the same femoral access, which was used for CPB.

He had significant bleeding for the next 24 hours on ECMO. He had persistent refractory hyperkalemia (serum potassium above 8 mmol/L). It did not respond to conventional measures to lower the potassium. CRRT was started in the ICU. Patient gradually worsened despite ECMO, IABP and heavy inotropes and expired on the 4th post-operative day.

Conclusion

1) Quick action is needed in saving patients with post infarct papillary muscle rupture and cardiogenic shock. In this patient, papillary muscle rupture and resultant acute severe MR could, unfortunately, be recognized only afer the patient was stabilized on ECMO, with his heart rate settling and with the help of TEE.

2) Multi-organ failure sets in very fast worsening the outcome and hence timely intervention is essential to salvage the myocardium and the patient.

3) The team should be prepared to take care of the very sick patient in the post-op period.

4) Family should be briefed about the situation and possible worst outcome.

In this patient, earlier ECMO might have helped to save the patient. In its absence at the referral unit, a mobile retrieval ECMO unit from our institution could have made it possible. Unfortunately such a mobile team was not existent at that time. Since then we have instituted the same.

Surgical ventricular restoration by means of Dor procedure is a surgical option in patients with coronary artery disease and post infarcular left ventricular aneurysm. This procedure gives a better outcome in those patients with poor left ventricular ejection fraction. Traditional cardiac surgical methods may have limited benefits in these types of patients. The Dor procedure excludes akinetic or dyskinetic portion of the anterior wall and septum, reshapes the LV with a stitch that encircles the transitional zone between normal contractile myocardium and aneurysmal scar tissue, and uses a patch to reestablish ventricular wall contour. By this technique, LV size and geometry is improved, reduces wall tension and enhances overall systolic function. Coronary bypass surgery is almost always performed with this procedure.

A 52-year-old American national was referred to cardiac surgery from outside the country with class IV symptoms. He was getting treatment for his hypertension and diabetes mellitus. His coronary angiogram showed left main stem stenosis and total occlusion of the LAD. Echocardiogram showed left ventricular aneurysm with LV clot, and no mitral regurgitation. His LV ejection fraction was 20%. He was scheduled for Dor procedure and CABG.

Surgical Procedure

Under cardiopulmonary bypass and cardioplegic arrest, the aneurysm was incised parallel to the LAD and clots were removed. A purse-string suture using 2-0 prolene was placed around the circumference of the scar at the transitional zone and tied down to determine size of the new ventricular opening. A Gore-tex patch was then sutured to the ventricular opening with interrupted 2-0 prolene suture. A second layer of continuous suture using 2-0 prolene is done. The edges of the ventricular free wall were then closed over the patch with a running 2-0 prolene supported with Teflon felt. The distal coronary anastomosis was done by saphenous vein to diagonal 1 and obtuse marginal 1. Left internal mammary was anastomosed to the proximal LAD. Proximal anastomosis was done to the ascending aorta with a side biting clamp. The patient was then weaned from CPB in a standard fashion. TEE was used to assess filling contractility and mitral valve function. He was electively put on IABP. Postoperative TEE showed improved LV contractility.

Discussion

The operative goal of the Dor procedure is to achieve complete coronary revascularization, reduce LV volume and restore its shape. The Dor procedure improves quality of life and survival in patients with left ventricular aneurysm. Also , complete revascularization is mandatory for a good outcome.

Classical myectomy is the gold standard therapy for patients with severely symptomatic hypertrophic obstructive cardiomyopathy. Extended myectomy provides a complete relief from dynamical obstruction and is most effective in the prevention of sudden cardiac death.

At Burjeel Hospital, seven patients underwent extended myectomy in the last 5 years.

Surgical technique

A detailed preoperative transesophageal echocardiogram done in the operation theatre. LVOT gradients were assessed both by TEE and simultaneous pressure recording of LV cavity and ascending aorta. All patients were operated while on cardiopulmonary bypass with cardioplegic arrest. A low transverse aortotomy was the incision of choice in all cases. No additional ventriculotomy done in any of these patients. The extent of the hypertrophy is assessed by visual inspection and digital palpation. The muscle mass suitable for myomectomy is clearly defined by classical incisions on the thickened LV septum. All precautions were taken not to damage the aortic annulus and the cusps. The incision was extended towards the LV apex to get complete relief of the obstruction. The excision of the muscle mass was also extended behind the insertion of the mural leaflet of the mitral valve. Almost always we tried to remove the muscle mass in total in single piece. LV cavity was thoroughly washed and inspected well. Both the papillary muscles were completely inspected and all hypertrophied trabeculae as well as hypertrophied papillary muscles were resected. Additional muscle band from the papillary muscle to the septum was found in one case and resected without damaging the papillary muscle.

Again, the LV cavity was assessed both visually and also by digital palpation. While coming off bypass, TEE helped to identify complete relief of the pressure gradient. Four patients had significant mitral regurgitation where leaflet plication was done with no significant post-operative mitral regurgitation. Post-operatively, none of these patients had aortic regurgitation or significant mitral regurgitation or septal perforation or conduction block. One patient had an iatrogenic perforation in the aortic leaflet, which was repaired by pericardial patch. All of them had significant reduction in the pressure gradient on postop TEE assessment. All these patients were regularly followed up by our cardiology team. Two of the patients required elective implantable defibrillator.

Conclusion

In our experience, with the technique of extended myectomy, sustained relief was achieved in surgically treated HOCM patients, without SAM or significant mitral regurgitation, at long-term follow-up. No patient had sudden cardiac death during follow-up till date. Referrals for HOCM surgical interventions are still restricted to severely symptomatic patients with significant LVOT gradients under basal conditions.

HOCM – PATIENT PROFILE

S.No	Date of Surgery	Age/Sex/Nationality	Symptoms
1	24/05/2017	31, Male, Bangladesh	Dyspnea, chest pain
2	10/01/2018	54, Female, Philippines	Exertional dyspnea, chest pain and dizziness3
3	10/03/2018	23, Female, Pakistan	Exertional dyspnea4
4	02/00/2019	55, Male, Syria	Exertional dyspnea5
5	03/10/2020	39, Female, India	Fatigue, dyspnea6
6	12/10/2020	34, Male, India	Exertional dyspnea7
7	17/07/2021	34, Male, Bangladesh	Exertional dyspnea

HOCM – INTRAOPERATIVE DETAILS

S.No	LVOT-AO Gradient, TEE	SAM	MR	Procedure	Additional Procedure
1	64 mmHg	Yes	Yes	Extended myectomy	MV repair2
2	80 mmHg	No	Yes	Extended myectomy	Aortic and mitral valve repair3
3	90 mmHg	Yes	Yes	Extended myectomy	MV replacement * outsidesurgeon4
4	100 mmHg	Yes	Yes	Extended myectomy	MV repair
5	65 mmHg	Yes	Yes	Extended myectomy	MV repair
6	140 mmHg	No	Yes	Extended myectomy
7	100 mmHg	No	Yes	Extended myectomy

Supravalvular aortic stenosis in adult: the three-patch technique:

Supravalvular aortic stenosis is a rare congenital malformation seen in pediatric age group as a result of an abnormal thickening of the aortic wall. This entity in adult age is further rare and we report a case of successful repair (Brom’s procedure) in an adult.

Prof. Brom worked in the UAE as the head of the department of cardiac surgery in Mafraq Hospital. During this time, he had his coronary bypass surgery done by Dr. Y. A. Nazer. Prof. Brom is considered as the father of pediatric cardiac surgery.

Introduction

Congenital supravalvular aortic stenosis (SVAS) is an uncommon anomaly presented as a narrowing situated at the level of the sinotubular junction. An early diagnosis is necessary in order to avoid future complications. Surgical treatment should ideally be performed in infancy to prevent early aortic valve degeneration, coronary artery pathology and left ventricular hypertrophy.

Case history

A 36-year-old male expatriate foreign national who presented with shortness of breath and chest pain of one-year duration. He had a detailed cardiology workup and his transthoracic echocardiogram showed supraaortic stenosis with post stenotic dilatation of the ascending aorta.

Preoperative transesophageal echocardiogram showed a supraaortic gradient more than 100 mmHg with mild aortic regurgitation.

Surgical technique

Under cardiopulmonary bypass and cardioplegic arrest, the aorta is transected just above the stenosis. Intraoperative findings were severe narrowing of the sinotubular junction with thick aortic valve. Aortic valve is tricuspid and normal leaflets. Left coronary osteum shifted more towards the left side.

Three longitudinal incisions were made in each sinus of Valsalva and rectangular shaped glutaraldehyde treated autologous pericardial patches were sutured. Care is taken to design the correct size of the patch. We have taken all precautions to avoid commissural extension and possible aortic regurgitation. An enlargement of the distal ascending aorta is also done with another piece of autologous pericardium (modified Brom’s procedure, four-patch technique).

Both coronary ostea were inspected and were found normal. Aorta closed in two layers and came off bypass in stable hemodynamics. Post-operative transesophageal echocardiography showed no gradient across the LVOT and aorta and no aortic regurgitation. The patient had a smooth post-operative recovery and discharged on seventh post-operative day. He is back to his normal activities.

Discussion

The three-patch technique, designed by Prof. Brom, offered a new concept for restoration of the aortic tract that continues to be the ideal model to repair supravalvular aortic stenosis both in pediatric and adult age group. There are multiple advantages in this technique. It provides a more symmetric reconstruction, less distortion of the aortic root and the ascending aorta. Glutaraldehyde treated autologous pericardium provides a normal thickness vascular tissue. This technique is easier than the other techniques and the individual patches can be designed in such a way that aortic root restoration will be optimal.