Coronary atherosclerosis is the commonest cause of cardiomyopathy in the United States, comprising 50–75 % of patients with heart failure. In addition, coronary disease may be present in patients with heart failure of other causes, and may sometimes be overlooked as a contributing factor [5]. There are two mechanisms for heart failure in this setting: a prior myocardial infarction (MI) followed by left ventricular dysfunction and remodeling; or hibernating myocardium due to chronic but potentially reversible ischemic dysfunction. Patients with ischemic heart disease may have heart failure from one or both of these mechanisms [6, 7].

Valvular heart disease is the primary cause of heart failure in perhaps 10–12 % of patients [8]. Furthermore, valvular dysfunction is a secondary or superimposed phenomenon in many cases of heart failure. An example of this is that there is almost always some degree of mitral or tricuspid regurgitation in patients with severe dilated cardiomyopathy [9].

Valvular dysfunction produces two forms of stress on the heart, volume overload and pressure overload. Both stressors result in increased cardiac afterload. Patients with valvular disease typically exhibit tremendous cardiac reserves. Due to the existence of various compensatory mechanisms, these patients can persist in an asymptomatic, well-compensated state until severe valvular and ventricular dysfunction have developed. In contrast, the patient developing acute valvular dysfunction without a period of gradual adaptation can rapidly succumb to severe heart failure [4].

Other potentially reversible conditions that can impair ventricular function and cause, or worsen, heart failure should also be evaluated. This includes, but is not limited to, hypertension, renovascular disease and drug therapy (a list of these drugs is shown in Table 3.2 [10]).

The presenting symptoms are important in determining the acuity of heart failure. Acute and subacute presentations (days to weeks) are characterized primarily by dyspnea at rest and/or exertion. Other specialized forms of dyspnea, such as orthopnea and paroxysmal nocturnal dyspnea, are also common. Right heart failure and tachyarryhthmias may be present resulting in symptomotology of hepatic congestion and palpitations respectively.

Chronic presentations (months) differ in that fatigue, anorexia, bowel distention and peripheral edema may be more pronounced than dyspnea. This is because pulmonary venous capacitance adapts to the chronic volume overload leading to less fluid accumulation in the alveoli, despite the increase in total lung water.

Although history alone is insufficient to make the diagnosis of heart failure [12, 13], it remains the single best discriminator to determine the acuity and rate of progression of heart failure. It also often provides important clues to the etiology of heart failure.

Physical examination can provide important information concerning the degree of volume overload, ventricular enlargement, pulmonary hypertension and reduced cardiac output.

A standard workup should include blood tests, a chest x-ray and electrocardiogram on all patients. The presence of pulmonary vascular congestion and cardiomegaly on chest x-ray support the diagnosis of heart failure. Chest x-ray is also useful for ruling out pulmonary disease in patients who present with dyspnea [14–16]. A normal electrocardiogram is unusual in patients with symptomatic systolic dysfunction (98 % negative predictive value) [17].

Echocardiography should be performed on all patients with new onset of heart failure. It is one of the most useful non-invasive tools available in aiding the management of heart failure. In addition to having a high sensitivity and specificity for the diagnosis of heart failure (80 % and 100 % respectively) [18], it can also detect other important findings. Regional wall motion assessment using dobutamine stress echocardiography may increase the ability to distinguish between ischemic and non-ischemic dilated cardiomyopathy [19]. This technique is also useful in predicting recovery of cardiac function [20, 21]. Echocardiography can also detect pericardial thickening, valvular heart disease, presence of thrombi, abnormal myocardial texture, chamber size and function, as well as measuring cardiac output using pulsed-wave Doppler.

Virtually all patients with unexplained heart failure should be evaluated for the presence of ischemic heart disease, as it is not an uncommon cause of dilated cardiomyopathy [5, 22]. This can be done using several techniques.

Non-invasive exercise testing not only provides information about the existence of ischemic heart disease, but can also be used for risk stratification and prognostic purposes. Measurement of the maximal oxygen uptake (VO₂max) provides an objective estimate of the functional severity of the myocardial dysfunction.

Coronary catheterization with angiography is indicated in patients with angina or a positive exercise stress test. However, even patients with a normal exercise stress test, who otherwise have unexplained heart failure, cardiac catheterization should strongly be considered. The ACC/AHA Committee on Coronary Angiography has published recommendations for the use of coronary angiography in patients with heart failure as shown in Table 3.4 [23]. It may also detect and grade underlying valvular heart disease, which may contribute to the symptomotology of heart failure.

The role of endomyocardial biopsy in discovering the etiology of dilated cardiomyopathy is not well defined. The yield for clinically useful information not obtainable without biopsy is low [22, 24, 25]. Hence, it should be reserved for patients with suspected systemic diseases known to affect the myocardium, including hemochromatosis, amyloidosis and sarcoidosis.

General indications for pacemaker implantation include patients with chronic atrial fibrillation who require atrioventricular nodal ablation for rate control and symptomatic bradycardias. The use of biventricular (BiV) pacemakers, termed resynchronization therapy, can improve symptoms in some patients with moderate to severe heart failure [44, 45]. As a result of this, BiV pacing has been approved by the USFDA as a treatment for moderate to severe heart failure. However, it has not determined when BiV pacing should be used. This is due to potential concerns, which include a small risk of serious complications occurring during implantation [44] and a lack of long-term data [11]. It is also not known if these devices should routinely be used with an implantable defibrillator.

In heart failure, both ventricular enlargement, resulting from damaged myocardium, and a ventricular aneurysm, which can develop after a heart attack, can compromise the heart’s ability to produce a strong contraction and leads to a decrease in cardiac output. Batista et al. first introduced the concept of surgically reversing this process, partial left ventriculectomy, for patients with NYHA class IV idiopathic dilated cardiomyopathies in 1996. They described a procedure that involved resection of normal muscle between both papillary muscles and extending from the apex to the mitral annulus. The mitral valve was either preserved, repaired or replaced depending on the amount of tissue removed. This restored the ventricle to a more normal volume/mass/diameter relationship [46]. The reduction in ventricular diameter, according to LaPlace’s law, results in decreased ventricular wall tension and possibly a more uniform pattern of contraction/relaxation, thus improving systolic performance [47, 48]. Although many patients initially improved markedly [47, 49–55], the perioperative mortality was high and many patients redilated [49, 56] with a high recurrence rate of symptomatic heart failure [51, 55, 57]. As a consequence, overall enthusiasm for the procedure has waned.

The newer surgical modalities for cardiomyopathy have benefited from the lessons learned in the treatment of left ventricular aneurysms. The Dor procedure is an approach to surgical reconstruction in the setting of postinfarction aneurysm formation [58]. Prior to the development of the Dor procedure, surgical treatment for postinfarction aneurysms involved removal of the aneurysmal area and reapproximation of the viable wall (endoaneurysmorraphy) in an attempt to restore left ventricular geometry. However, this approach has not been found to improve left ventricular performance [59]. In the Dor procedure, also called endoventricular circular patch plasty (EVCPP), a purse string stitch is placed around the circumference of the non-viable scarred aneurysm to minimize the excluded area. The size of the scar is more important than whether it is akinetic or dyskinetic. Larger scars may yield a greater improvement in left ventricular ejection fraction (LVEF) but have a significantly higher perioperative mortality rate (12 % versus 2.2 % for a small scar). The residual defect is then covered with a Dacron, pericardium or an autologous tissue flap. This operation shortens the long axis, leaving the short axis length unchanged, producing an increase in ventricular diastolic sphericity while the systolic shape becomes more elliptical [60, 61]. This results in a more normal geometry and improved systolic performance. The overall operative mortality in the first patients who underwent the Dor procedure was 8 %. Operative mortality was higher when surgical repair was performed urgently 16.3 % compared to 6.2 % when it was planned [62]. The overall improvement of LVEF was maintained at 1 year. Also, there was a reduction in the end-diastolic volume index and symptomatic heart failure status improved by 92 % [63].

A modification of the Dor procedure, surgical anterior ventricular endocardial restoration (SAVER), consists of exclusion of non-contracting segments in the dilated remodeled ventricle after an anterior MI. The efficacy of this approach was evaluated in the multicenter RESTORE trial; 89 % also underwent bypass grafting and 26 % had mitral valve repair or replacement. It revealed a significant reduction of left ventricular end-systolic volume index and an increase in LVEF from 29 to 39 % [62]. Furthermore it has been noted that the Dor procedure ameliorates mitral regurgitation in a majority of cases even in the absence of associated mitral valve procedures, probably due to the reduction in the size of the ventricle and improved orientation if the papillary muscles [64]. The choice of the beating heart approach or continuous aortic cross-clamp plays little to no role in postoperative outcome. Furthermore, the added ischemic time associated with continuous cross-clamp is outweighed by the potential benefits [65]. Cope et al. conducted a cost comparison of heart transplantation versus alternative operations for cardiomyopathy in 2001 [66]. This study compared the cost and survival among patients that have undergone heart transplantation, isolated coronary artery bypass grafting (CABG), mitral valve replacement and the Dor procedure. It was noted that the total cost of heart transplantation, which included the procurement costs, was significantly higher among the heart transplant group. The cost was comparable among the other three groups. The operative survival was similar among the four groups. Thus, ventricular reconstructive surgery is a practical alternative to heart transplantation in the select patients. Recommendations for the indications for ventricular reconstructive surgery have been proposed in a 2002 review as shown in Table 3.5 [64].

Mitral regurgitation (MR) is often a complication of end-stage cardiomyopathy. It can result from dilatation of the mitral annular-ventricular apparatus with altered geometry and ischemic papillary muscle dysfunction [67, 68]. Although the teaching for decades has been that closing off the mitral valve leak removes the “pop-off” valve and is associated with an unacceptable surgical death rate, it is important to keep in mind that the regurgitation of blood into the left atrium leads to a cycle of more volume overload, which can lead to progressive annular dilatation, worsened MR, and more severe symptoms of congestive heart failure [69]. Severe MR in patients with ischemic cardiomyopathy is a difficult management issue. Not only do these patients have worsened symptoms, but they also have an increased death rate. The reported 1-year survival rate for medical therapy in this subset of patients is less than 20 % [70]. Among patients with significant (>2+) secondary MR, mitral valve repair should be considered in NYHA class III/IV patients with dilated cardiomyopathies [3]. Several studies have shown that mitral valve repair in patients with end-stage cardiomyopathy is not only feasible but also improves ventricular function and overall survival [70–75]. If mitral valve repair is not possible, it is essential that mitral valve replacement be performed with retention of the subchordal attachments. Preservation of both the anterior and posterior chordal attachments to the papillary muscles helps to maintain normal ventricular geometry and function following mitral valve replacement [76–79]. Most of these studies, however, have dealt with mitral valve repairs in patients with non-ischemic dilated cardiomyopathy.

Ischemic MR seems to be more complex. Often the posterior leaflet becomes functionally restricted owing to ventricular enlargement. Patients with severe MR secondary to ischemic cardiomyopathy have two separate pathophysiologies that not only augment pump failure, but also need to be each dealt with separately surgically. The surgical management of these patients, particularly those requiring both mitral valve surgery and concomitant CABG, has traditionally been associated with an increased surgical risk [80]. Clearly these patients represent a substantial increased surgical risk compared with patients undergoing either CABG or mitral valve surgery alone [72]. Fortunately, with the advances in myocardial protection and surgical techniques, some of these more difficult patients are now not only at a lower surgical risk, but also are experiencing improvements in their symptomatic status. A more recent study concluded that concomitant CABG and mitral valve repair compare favorably to both CABG alone and cardiac transplantation, thus offering a reasonable alternative for this patient population [81].

Heart failure resulting from coronary artery disease (CAD) is usually due to MI and subsequent ventricular remodeling. When the ischemia is chronic, the recoverable or viable myocardium is termed “hibernating” but when the insult is transient, that myocardium is described as “stunned”. The impaired left ventricular function in these patients is not entirely an irreversible process, if hibernating myocardium is in part responsible to the decline in myocardial function. About 40 % of segments involved in MI may subsequently recover, either spontaneously or after revascularization. Also, LVEF may improve markedly, and even normalize, in subsets of patients following successful revascularization [82, 83]. Patients with documented viability by thallium perfusion imaging, PET scanning or dobutamine echocardiography had significant 80 % reduction in annual mortality with revascularization. There was a direct relationship between severity of left ventricular dysfunction and magnitude of benefit. In contrast, there was no difference in outcome with revascularization or medical therapy in patients without viability [7].

Surgical revascularization for patients with ejection fractions of <20 % to recruit hibernating myocardium is now becoming commonplace [84]. These patients are generally sicker with more preoperative risk factors. However, despite increased hospital mortality of about 4–6 %, they enjoy 90 % 1-year survival and 64 % 5-year survival [85]. Patients with ischemic cardiomyopathy, evidenced by viable myocardium, and bypassable vessels can be revascularized with permissible risk, achieving 88 % perioperative survival with 72 % of patients alive at 1 year. These results are reproducible and have been reported by different authors [86–89].

It is commonly believed that patients with both ischemic cardiomyopathy and significant left ventricular dilatation should undergo transplantation secondary to poor outcome after CABG [90]. However, Aziz and associates [91] addressed this issue by comparing the outcomes of cardiac transplantation between patients with ischemic cardiomyopathy and those with idiopathic cardiomyopathy. Although the operative mortality between the two groups was essentially the same (11.2 % and 10.6 % respectively), the 10-year survival was remarkably different (39 % and 80 % respectively). Hence, the decision to operate on patients with severely depressed left ventricular function is not straightforward. There are excellent short-term results with CABG alone but inferior mid-term results compared with CABG plus ventricular remodeling [92].

The usefulness of angioplasty depends upon the pattern and extent of arterial narrowing. Angioplasty is often recommended over bypass surgery when arterial narrowing is mild or moderate and when only one or two coronary arteries are narrowed. It is more effective in patients who do not have diabetes mellitus. Patients with diabetes appear to have a greater benefit from CABG, especially in the setting of multivessel, multilesion or severe CAD [93].

Recommendations for revascularization for patients with native-vessel CAD by the ACC/AHA/ACP-ASIM are shown in Table 3.6 [94]. These recommendations were ratified again in the 2002 update.