Successful surgical management for aortic and mitral stenosis in homozygous familial hypercholesterolemia: a case report

A 72-year-old woman with a known history of homozygous familial hyperlipidemia (IIa) was referred to our hospital for an operation necessitated by aortic and mitral stenosis and paroxysmal atrial fibrillation. Computed tomography and cardiac catheterization revealed a heavily calcified aortic root and mitral annulus as well as a high-grade stenosis of the left anterior descending and right coronary arteries. Double aortic and mitral valve replacement concomitant with replacement of ascending aorta, maze III procedure, and coronary artery bypass were performed. Temporary hypothermic arrest was employed to reduce the risk of cerebral emboli.     

Current surgical treatment for acquired valvular heart disease

We discuss the current status of surgical treatment for acquired valvular heart disease. Mitral valve repair for organic and functional mitral regurgitation is the first choice instead of valve replacement. It is important that surgery for functional mitral regurgitation restores the geometry of the left ventricle and mitral valve. The reduction of mitral valve tethering for functional mitral regurgitation is a current topic of discussion. At present, the surgical procedure for both aortic stenosis and aortic regurgitation is valve replacement in most cases, although aortic valve repair has been attempted for aortic regurgitation in recent years. The early results of aortic valve repair are excellent, but the long-term results have not been clarified. The durability of valve repair in both the mitral and aortic position is a future issue and it may be improved by revising the indications for valve repair and using new surgical techniques.     

The mitral hooks: visualization of mitral valve made easy

Historically the mitral valve has been exposed through a variety of approaches from the standard left atriotomy to cardiac autotransplantation. Regardless of the approach, adequate exposure to the mitral valve is crucial to a successful valve repair or replacement. We describe a simple and effective way of bringing the mitral valve into view through a standard left atriotomy with the use of specially designed and modified mitral hooks. The mitral hooks provide excellent unobstructed view of the mitral valve, which is suitable for repair and replacement.     

Combined techniques for double valve replacement in the infant

A 6-month-old female infant was seen with heart failure secondary to severe aortic and mitral regurgitation. As a neonate the infant had undergone an aortic valvotomy for congenital aortic stenosis. Subsequently the infant had aortic and mitral regurgitation with an infarcted papillary muscle. Double valve replacement was carried out with the St. Jude valve. The first approach was by the Manouguian procedure with extension of the aortotomy out between the left coronary cusp and the noncoronary cusp. The posterior mitral apparatus was resected, and a 19-mm St. Jude aortic valve was sewn into the mitral position. Because the enlarged aortic valve annulus was still inadequate to accommodate a 19-mm St. Jude valve, a Konno procedure was carried out to enlarge the aortic ring anteriorly. Atrial, septal, and aortic repair and right ventricular outflow tract reconstruction were carried out with bovine pericardium. Bypass was carried out with standard techniques of hypothermia, aortic cross-clamping, and cardioplegia. Postoperative anticoagulation therapy was initially with aspirin and dipyridamole (Persantine); however, clotting of the mitral prosthesis necessitated treatment with urokinase and heparin, which completely resolved the clot. Sodium warfarin (Coumadin) therapy was then begun. One year postoperatively, the child is developing normally.     

Critical subaortic stenosis in a newborn caused by accessory mitral valve tissue

A 2-week-old newborn girl underwent successful surgery in our clinic for critical subaortic stenosis caused by accessory mitral valve tissue, which, because of excessive growth, protruded into the left ventricular outflow tract. The preoperative pressure gradient below the aortic valve was 80 mm Hg. The operation consisted of resection of the accessory tissue through a combined aortotomy and atriotomy approach without residual pressure gradient and mitral valve incompetence. This approach is recommended to ensure that accessory tissue is removed without damaging the mitral valve.     

Manouguian double-valve replacement in a 6-year-old girl

A 6-year-old girl with a diagnosis of aortic regurgitation with stenosis and mitral regurgitation because of short chordae was referred to us for surgery. Echocardiography revealed that the aortic and the mitral annular diameters were 16 and 23?mm, respectively. The Ross procedure and mitral valvuloplasty were scheduled. During the procedure, we were concerned that aggressive mitral valvuloplasty might result in mitral stenosis. We therefore converted the procedure to double-valve replacement using the Manouguian technique because it was necessary to enlarge both the aortic and mitral annuli. In children, the mitral prosthetic valve in Manouguian technique may override aortic annulus resulting in left ventricular outflow tract obstruction (LVOTO). Thus, it is important to decide the mitral prosthetic valve size. Measurements of both annuli showed 15 and 21?mm in aortic and mitral positions, respectively. Size #18 ATS AP mechanical valve (ATS Medical, Inc., Minneapolis, MN, USA) and size #23 ATS mechanical valve were implanted. We successfully performed two sizes up in the aortic position and one size up in the mitral position avoiding complications such as coronary orifice obstruction and LVOTO. To our knowledge, this is the youngest patient who underwent double-valve replacement by the Manouguian technique.     

The transseptal approach to the mitral valve during multivalvular surgery

Abstract Aim: We investigated the short and mid‐term outcome of the transseptal approach to the mitral valve during multivalvular surgery. Methods: Within a three‐year period ending in May 2010, we used the transseptal approach in performing mitral valve surgery in 62 patients. Procedures performed were: mitral valve replacement and tricuspid annuloplasty in 40 patients, both aortic and mitral valve replacement with tricuspid annuloplasty in 13 patients, mitral valve and tricuspid valve replacement in eight patients and mitral valve repair and tricuspid annuloplasty in addition to coronary artery bypass surgery in one patient. Results: There were no complications associated with the transseptal approach. There were no conduction abnormalities, nor were there any procedure‐related deaths. Conclusion: We conclude that use of the transseptal approach for mitral valve operations is simple and safe in patients necessitating right atriotomy for concomitant procedures. (J Card Surg 2011;26:472‐474)     

Repair of injured right inferior pulmonary vein during mitral valve replacement

During mitral valve surgery right pulmonary veins injury, subsequent to excessive traction (for better exposure of the mitral apparatus), is often unavoidable. This is more likely in patients with small left atrium. This common complication may cause severe intraoperative bleeding, while its surgical repair may lead to complications such as late stenosis or obstruction of the pulmonary veins. This injury should be early detected, before left atriotomy closing, and it is suggested to be repaired using a patch so as to avoid any possible late constriction.     

Aortic and mitral valve replacement for calcified valvular annulus in a dialysis patient; report of a case

A 77-year-old man on hemodialysis was admitted to our hospital due to heart failure. Echocardiography showed aortic valve stenosis and regurgitation, mitral valve stenosis and regurgitaion, and tricuspid valve regurgitation. Catheter examination revealed severe calcification at aortic valve and mitral valve including their annulus. At the operation, the calcifications of the aortic and mitral valvular annulus was removed using a cavitron ultrasonic surgical aspirator (CUSA). Reconstructions of the defect of the posterior part of the mitral annulus and of the aortic annulus at the site of the left coronary cusp were achieved by patch technique using autologous pericardium. Aortic and mitral valve replacement and tricuspid valve annuloplasty were performed. The postoperative course was uneventful. Operative technique to remove calcification from valvular annulus using CUSA and reconstruct of the defect of the annulus with autologous pericardium is a very useful technique to prevent left ventricular rupture, perivalvular leakage and any other complications.     

Combined coronary artery bypass, mitral valve plasty, and abdominal aneurysmectomy in an 80-year-old patient: Report of a case

An 80-year-old man suffering from angina on exertion due to stenosis of the left main coronary artery, heart failure due to mitral valve regurgitation, and an abdominal aortic aneurysm (AAA) was successfully operated on with simultaneous surgical procedures. A coronary cineangiography revealed 90% stenosis of the left main coronary artery in segment 5, and 99% and 90% stenosis in segments 2 and 4AV, respectively, of the right coronary artery. Left ventriculography and aortography showed moderate mitral valve regurgitation and the presence of a fusiform-shaped AAA with a maximum diameter of 6 cm. It was thought that insertion of an intraaortic balloon pump (IABP) would prove difficult due to AAA; therefore, simultaneous surgery combining triple coronary artery bypass grafting (CABG), mitral valve plasty, and prosthetic replacement of the AAA was undertaken. The patient's postoperative course was uneventful, and subsequent angiography showed good patency of all coronary bypass grafts and the abdominal prosthesis, along with the disappearance of mitral regurgitation. This patient's clinical course suggests that an extended surgical procedure is effective for the treatment of complicated cardiovascular disease, even in very elderly patients.     

A case report of translocation method for active infective aortic valve endocarditis with aortic root abscesses]

We experienced a case of 51-year-old woman who underwent emergency aortic valve replacement by translocation method for active infective aortic valve endocarditis with aortic root abscesses. Postoperative course was complicated as the following. Three days later, the perforation of noncoronary sinus of Valsalva into the right atrium was noted and she developed progressive heart failure due to the massive left-to-right shunt. The second operation was performed immediately for the patch closure of the perforation through the right atriotomy. Two months later, unstable angina appeared because of the stenosis of the vein graft to the left coronary artery, leading to the emergency third operation in which LITA was placed to the left anterior descending artery. In spite of these complications she recovered gradually and she was discharged 6 months after the first operation. She is now doing well in NYHA class 2. Translocation method is quite useful for such a case of the aortic valve endocarditis with periannular abscesses in whom conventional valve replacement is supposed to be impossible, but the long durability of this type of the repair is unknown. Careful follow-up of the patient is mandatory.     

Isolated left ventricular ischemia after the Norwood procedure

Aortic atresia is the most severe variant of hypoplastic left heart syndrome (HLHS), and has been associated with significant mortality after stage I palliation. Coronary artery abnormalities are more prominent in this group of patients, especially in the presence of a patent mitral valve. Herein, we describe a case of isolated left ventricular ischemia after the Norwood procedure in a neonate with hypoplastic left heart syndrome, left ventricular hypertrophy, mitral stenosis, aortic atresia, and anomalous left coronary artery.     

Modified cavoatriotomy for combined PAPVC repair and maze procedure

In patients with partial anomalous pulmonary venous connection (PAPVC) to the superior cavoatrial junction, the standard right-sided left atriotomy does not allow sufficient access to the mitral valve and the left atrium. And the injury and traction of the sinus node and sinus node artery should be avoided for prevention of the cardiac rhythm disturbance after operation. We herein report a useful approach to repair the sinus venosus atrial septal defect with PAPVC of the right pulmonary veins to the superior cavoatrial junction in patients also requiring mitral valve replacement and the maze procedure.     

Selective antegrade cardioplegic perfusion complicated by left main stem dissection

Selective antegrade coronary artery perfusion is a commonly used procedure to obtain myocardial preservation during cardiac surgery. This report describes a patient operated for severe aortic valve stenosis and insufficiency, mitral valve and tricuspid insufficiency. Cardioplegia was administered by selective antegrade coronary artery blood perfusion. Antegrade blood cardioplegia was complicated by dissection of the left coronary main stem. The dissection induced a myocardial infaction and the patient finally died due to heart failure.     

Ruptured aneurysm of sinus of Valsalva. Long-term postoperative follow-up

Ruptured sinus Valsalva aneurysm was repaired in 13 patients (mean age c. 33 years). Dyspnea, chest pain, fatigue and palpitation were the most common symptoms and systodiastolic murmur, cardiomegaly and pulmonary congestion the most pertinent clinical findings. The pulmonary-to-systemic flow ratio averaged c. 2.5. Associated cardiac anomalies were ventricular septal defect, aortic or mitral regurgitation, aortic coarctation or subvalvular stenosis, tetralogy of Fallot (altogether 8 cases). The origin of the fistula was the noncoronary, right coronary or left coronary sinus (5, 4 and 3 cases) or was not identifiable (1 case). Rupture occurred into the right atrium (6 cases), right ventricle (6) or pulmonary artery (1 case). Repair was undertaken through aortotomy (6 cases), right ventriculotomy (2) or right atriotomy (1) or through aortotomy + right ventriculotomy or atriotomy (4). In one case aortic valve replacement was performed. All survived the operation. Follow-up averaged 9.6 years. Recurrent fistulation, though with small shunt, was found in two cases. Combined two-dimensional and Doppler echocardiography revealed minor cardiac abnormalities in most patients, particularly aortic regurgitation. All the patients were in NYHA function class I or II.     

Iatrogenic left main and proximal right coronary artery stenoses after aortic valve replacement.

Iatrogenic left main coronary artery stenosis after aortic valve replacement is an infrequent but potentially life-threatening complication. A 44-year-old woman who had normal coronary arteries documented by preoperative coronary angiogram, and who developed severe stenosis of the left main coronary artery and subtotal occlusion of the proximal right coronary artery after aortic and mitral valve replacements is presented. Coronary lesions were clinically manifested 4 months after the first operation. Accurate diagnosis was confirmed by repeat coronary angiography. She underwent successful coronary artery bypass grafting.     

Combined surgery for valvular and ischemic heart disease

Combined coronary artery bypass (CAB) and valve surgery is one of the most challenging surgical procedures, but the operative results have improved over the years. We discuss several important points in combined surgery. The first point is cardioplegia, which should be perfect in such complex operations. Sufficient antegrade cold blood cardioplegia should be used in combined CAB and mitral valve surgery. Continuous retrograde cardioplegia is required in CAB and aortic valve surgery. The second point is the prosthesis and grafts. A mechanical prosthesis and arterial grafts should be used in younger patients, while a bioprosthesis and vein grafts with a left internal thoracic artery graft should be used in older ones. Finally, the choice of valve repair or replacement must be considered in mitral surgery with CAB. Valve repair is the choice in patients with mitral prolapse due to chordal rupture, because a perfect repair can be achieved using a well-known procedure. In cases in which repair appears difficult, replacement must be carried out as soon as possible. In mitral valve replacement the continuity between the papillary muscles and the mitral ring must be preserved for good left ventricular performance.     

Repair of anomalous left main coronary artery arising from the pulmonary artery in infants: long-term impact on the mitral valve

Background. Infants presenting with anomalous left coronary artery off the pulmonary artery (ALCAPA) are generally in heart failure and often have significant mitral valve regurgitation (MR). Although establishing a dual coronary circulation is the procedure of choice, there remains controversy as to how the mitral valve is handled.

Methods. We reviewed our experience with this lesion at St. Louis Children’s Hospital. Over the past 15 years, 17 infants under 18 months of age have undergone repair, with all but one being treated with reimplantation of the left coronary artery into the aorta; the other underwent the Takeuchi procedure (intrapulmonary artery baffle) and was excluded from this evaluation. The average age and weight at operation were 0.5 ± 0.3 years and 6.1 ± 1.9 kg, respectively. All presented with varying degrees of heart failure and 9 patients also had either moderate or severe MR.

Results. There was one early and no late deaths after reimplantation of the left coronary artery. The left ventricular function postrepair improved from a preoperative shortening fraction of 0.19 ± 0.09 to 0.34 ± 0.08 (p < 0.01). Moderate or severe MR was present in 2 patients postoperatively, and both developed significant obstruction in the left coronary artery postoperatively as well. Both underwent mitral valve repair and revascularization of the left coronary artery.

Conclusions. Excellent results can be obtained in the treatment of this very high-risk group of patients. Mitral valve repair is not generally necessary at the time of the initial operation. However, should MR recur or persist late, it may herald the presence of a coexistent, significant coronary stenosis. Cardiac catheterization should be performed to assess the patency of the left coronary artery before performing mitral valve surgery.     

Inverted L-shape sternotomy as a minimally invasive approach

We report a successful treatment of the complete papillary muscle rupture occurring 16 months after coronary artery bypass grafting (CABG). A 57-year-old man was admitted for the sudden onset of chest pain and cardiogenic shock. Emergency cardiac catheterization revealed severe mitral regurgitation and total occlusion in the right coronary artery, which was successfully revascularized by percutaneous coronary intervention under intra-aortic balloon pumping. The right internal thoracic artery grafted to the left anterior descending artery in the previous CABG was functioning well. An echocardiogram distinctly indicated the ruptured head of the papillary muscle. Since an emergency operation revealed complete rupture of the posterior papillary muscle, mitral valve replacement was carried out through an inverted L-shape sternotomy with T-shape left atriotomy. Our case indicates that the inverted L-shape sternotomy was a useful approach to preserve the function of grafts, and that T-shape left atriotomy offered a good exposure of the mitral valve in the limited surgical field.     

Mitral Stenosis in Association with Aortic Valvular Disease

In a series of 47 consecutive patients with pure mitral stenosis in association with aortic valvular disease, 25 patients underwent closed mitral commissurotomy in addition to aortic valve replacement, whereas combined aortic and mitral valve replacement was performed in 22 patients. The pathology of the stenosed mitral valve and resultant left atrial stasis were more pronounced in the latter group. Valve replacements were performed under generalized hypothermia to 30°C and selective coronary artery perfusion. The advantages and disadvantages of commissurotomy in comparison with mitral valve replacement were evaluated. Commissurotomy with aortic valve replacement involved a markedly higher mortality rate, which was closely related to early and late complications from the closed mitral commissurotomy per se. The reduced risk of thrombo-embolism following preservation instead of replacement of the mitral valve hardly outweighs this excessive mortality. The late haemodynamic improvements, although slight, were definite and similar in the two groups of patients. Diminished pressure levels in the left atrium and pulmonary artery were observed both at rest and during exercise in all the 19 patients who underwent recatheterization. Three patients showed signs of slight mitral restenosis (or residual stenosis), while another required mitral valve replacement due to mitral restenosis with incompetence. Commissurotomy remains clearly indicated if the commissures split up completely and the valve leaflets, chordae tendineae and papillary muscles are in unquestionably good condition. Unfortunately, this type of mitral stenosis, particularly in association with aortic valvular disease, is seldom encountered in Sweden nowadays. Mitral valve replacement seems to be mandatory when surgery is performed for restenosis and if uncertainty exists about the immediate result of commissurotomy.