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Ann Thorac Surg 2002;74:946-955
© 2002 The Society of Thoracic Surgeons

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Review

Anomalous origin of the left coronary artery from the pulmonary artery: collective review of surgical therapy

^a Division of Cardiovascular-Thoracic Surgery, Children’s Memorial Hospital, Chicago, Illinois, USA
^b Department of Surgery, Northwestern University Medical School, Chicago, Illinois, USA

* Address reprint requests to Dr Dodge-Khatami, Division of Cardiovascular-Thoracic Surgery, Academic Medical Center, Room G4-229, Postbus 22660, 1100 DD Amsterdam, The Netherlands
e-mail: a.dodgekhatami{at}amc.uva.nl

	Abstract

Top Abstract Introduction Physiology Classification Diagnosis Surgical evolution and current... Surgical results Operative risk factors Myocardial scarring and mitral... Mechanical circulatory support Ultrastructural residual and... Conclusions References

 
Anomalous origin of the left coronary artery from the pulmonary artery is an extremely rare but potentially fatal congenital coronary anomaly. Prompt surgical reestablishment of a two-coronary system on diagnosis yields excellent results and allows progressive and nearly total myocardial recovery. Follow-up of all patients is required to assess the adequacy of repair and to exclude ongoing or recurrent myocardial insult.

	Introduction

Top Abstract Introduction Physiology Classification Diagnosis Surgical evolution and current... Surgical results Operative risk factors Myocardial scarring and mitral... Mechanical circulatory support Ultrastructural residual and... Conclusions References

 
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly, first described by Brooks [1] in 1886. It is usually seen as an isolated lesion [2, 3] and is present in one of 300,000 live births (0.25% to 0.5%) [2, 4, 5]. It represents one of the most common causes of myocardial ischemia and infarction in children and if left untreated, results in a mortality rate of up to 90% within the first year of life [6]. Immediate surgical correction on diagnosis with the aim of restoring a two-coronary system circulation is the current standard in patients with ALCAPA [3, 7, 8]. Although early diagnosis and prompt surgical intervention allow excellent results and gradual myocardial recovery, the possibility of persistent or recurrent substructural myocardial damage and subclinical ischemia remains, thus justifying regular postoperative follow-up.

The surgical history of ALCAPA along with current diagnostic strategies, surgical techniques, results, and modalities of follow-up is reviewed.

	Physiology

Top Abstract Introduction Physiology Classification Diagnosis Surgical evolution and current... Surgical results Operative risk factors Myocardial scarring and mitral... Mechanical circulatory support Ultrastructural residual and... Conclusions References

 
The original clinical spectrum of ALCAPA was first reported in 1933 [9]. Also known as the Bland-White- Garland syndrome, myocardial ischemia results in the typical presentation in infants of failure to thrive, profuse sweating, dyspnea, pallor, and atypical chest pain on eating or crying. In adults, malignant arrhythmias leading to sudden death may be the first manifestation of the anomaly [10].

Edwards [11] is credited with the first pathophysiological explanation of coronary artery dynamics in patients with ALCAPA. During the neonatal period, high pulmonary vascular resistance and resultant pulmonary artery (PA) pressures ensure antegrade flow from the PA into the anomalous left coronary artery. As the pulmonary vascular resistance gradually decreases, there is less antegrade flow to the left coronary artery, with eventual reversal of flow and left-to-right shunting into the PA. Consequently, a "coronary steal" results [3, 11], and left ventricular (LV) myocardial perfusion becomes dependent on intercoronary collaterals from an enlarged right coronary artery (RCA) [11, 12]. Historically, the rapidity of this sequence led investigators to classify patients on the basis of their respective survival patterns into two subsets of coronary circulations: the infantile or the adult type [12].

The infantile type of circulation has little or no coronary collateral development, a condition leading to the early onset of severe myocardial ischemia, LV dysfunction and dilatation, and mitral regurgitation (MR) from papillary muscle ischemia, dilatation of the annulus, or both [11, 13]. Without surgical correction, rapid death results within weeks to months after birth. In the adult type, accounting for only 10% to 15% of patients [13], intermediate to long-term survival is aided by a large dominant RCA with extensive intercoronary collaterals, as well as a restrictive opening between the ALCAPA and the PA [9, 12, 14, 15]. These patients can remain asymptomatic until adulthood, despite ongoing subclinic myocardial ischemia. In this subset of patients, there is an estimated 80% to 90% incidence of sudden death at a mean age of 35 years [13–17].

Smith and associates [18] described histological anomalies in both the left coronary artery and the RCA that could either explain the ischemic picture or derive from it. In a study of 14 hearts examined post-mortem, proximal or distal hypoplasia of the left coronary artery or a combination of both was observed at gross inspection, along with hypoplasia of the media and reduced presence of intimal cushions, findings suggesting developmental immaturity. An abnormal shape of the right coronary orifice, ostial stenosis, and hypoplasia of the RCA were noted in those specimens with minimal intercoronary collaterals [18].

Medical management is associated with dismal survival; reported mortality rates range between 45% and 100% [6, 13, 14, 19]. Many of these deaths occurred within months of the diagnosis in association with protocols calling for deferral of operation until 1 year of age [20, 21]. We currently recommend surgical intervention at the time of diagnosis (i.e., within 1 day to 2 days of echocardiographic confirmation), a view supported by others who also emphasize the importance of early operation [3, 7, 8, 14, 22–24]. On the basis of long-term evidence of irreversible myocardial damage in untreated patients [13, 25], it has become obsolete to divide patients with ALCAPA into two different groups. Even in adults with ALCAPA who have not had an operation, malignant ventricular arrhythmias have disappeared after reestablishment of a two-coronary system [10], which if untreated, would represent the physiological substrate for sudden death. These findings indicate the severity of the ischemic process regardless of symptomatology and justify surgical correction as soon as the diagnosis is made, regardless of age or degree of intercoronary collateralization [3, 16, 20, 23, 26–28].

	Classification

Top Abstract Introduction Physiology Classification Diagnosis Surgical evolution and current... Surgical results Operative risk factors Myocardial scarring and mitral... Mechanical circulatory support Ultrastructural residual and... Conclusions References

 
Few classifications of ALCAPA exist, and those that do are commonly descriptive in regard to the origin of the anomalous left coronary artery. In a general classification of coronary artery anomalies, Ogden [29] defined ALCAPA as a major coronary anomaly, referring to a coronary origin other than from the aortic root. More recently, Smith and associates [18] proposed a nomenclature relating to the sinus of origin of the anomalous coronary artery. Using the standard designation 1 for the sinus of origin of the RCA and 2 for the sinus from which a normal left main coronary artery would originate, the facing sinuses of the pulmonary trunk are numbered inversely (Fig 1), respectively 2 and 1. The sinus from which the ALCAPA originates is therefore defined from the standpoint of the nonfacing sinus of the pulmonary trunk which looks toward the aorta. Most commonly, the ALCAPA takeoff is from the right hand pulmonary sinus (sinus 1 of PA), which faces the aortic sinus where the left main coronary artery normally originates (sinus 2 of aorta). More rare origins of the ALCAPA include the left-hand pulmonary sinus, above the commissure between the right-hand and nonfacing sinuses, and the posterior aspect of the right or the left PA near its origin [18]. Incorporating Smith’s classification, an inclusive nomenclature system including all possible variations of ALCAPA origin is described in the Congenital Heart Surgery Nomenclature and Database Project [30].

View larger version (23K): [in this window] [in a new window]   Fig 1. Normal coronary distribution (inset), and sinus numeration in anomalous left coronary artery from the pulmonary artery (ALCAPA) as viewed from above. (Ao = aorta; L = left; LMCA = left main coronary artery, NF = nonfacing sinus; PA = pulmonary artery; R = right; RCA = right coronary artery; 1, 2 = sinuses of pulmonary valve.) (Reprinted from Dodge-Khatami A, Mavroudis C, Backer CL. Congenital Heart Surgery Nomenclature and Database Project: anomalies of the coronary arteries. Ann Thorac Surg 2000;694: Suppl S270–97); by permission of Elsevier Science Inc.)

	Diagnosis

Top Abstract Introduction Physiology Classification Diagnosis Surgical evolution and current... Surgical results Operative risk factors Myocardial scarring and mitral... Mechanical circulatory support Ultrastructural residual and... Conclusions References

Johnsrude and associates [33] defined electrocardiographic criteria distinguishing ALCAPA from myocarditis or dilated cardiomyopathy. They found a Q wave depth of more than 3 mm, a Q wave width greater than 30 ms, and a QR pattern in at least one of leads I, aVL, V₅–V₇, to be present in 100% of the ECGs of patients with ALCAPA. The absence of Q waves in leads II, III, and aVF was also universal. With the model ALCAPA = 12d + 24s-w, where d = Q wave depth in aVL, s = ST segment amplitude in aVL, and w = Q wave width in lead I, ALCAPA was correctly diagnosed in 98% of their patients, conferring a sensitivity of 100%, a specificity of 96%, and a positive predictive value of 85%. Using electrocardiographic criteria together with two-dimensional echocardiography, Chang and Allada [34] described a scoring system based on logistic regression, that allowed them to distinguish between dilated cardiomyopathy and ALCAPA. Including QT patterns in lead aVL, echocardiographic evidence of a RCA to aortic annulus ratio greater than 0.14, increased papillary muscle echogenicity, and Doppler color flow through the left coronary artery, diagnosis of ALCAPA was achieved with 100% sensitivity and 91% specificity.

Diagnosis can sometimes be achieved on the basis of echocardiography alone [3, 17, 35, 36] if it reveals a grossly enlarged RCA and a dilated left ventricle with global hypokinesia. Pulsed and color-flow Doppler can demonstrate reversal of flow from the ALCAPA into the PA, which constitutes a left-to-right shunt. In older patients, abundant high-velocity flow may be seen in the interventricular septum, as a result of intercoronary collaterals. Two-dimensional echocardiography can equally visualize the actual anatomical origin of the ALCAPA and assess the degree of mitral insufficiency.

Coronary angiography or ventriculograms are not routinely necessary in the current era [36]. When the diagnosis of ALCAPA is suspected but not visualized by echocardiography, coronary angiography is indicated to exclude another coronary anomaly potentially responsible for myocardial ischemia [3, 36], to define coexisting intracardiac defects, or to definitively exclude ALCAPA before settling for the diagnosis of idiopathic dilated cardiomyopathy.

Myocardial viability studies such as dobutamine echocardiography, thallium and sestamibi single-photon emission computed tomographic myocardial perfusion imaging, and positron emission tomography using nitrogen 13-labeled ¹³N-ammonia or carbon-13-labeled acetate, are used in the assessment of hibernating myocardium [37]. Preoperatively, however, these would influence surgical strategy only in patients with massive infarction or aneurysmal tissue where improvement through coronary artery revascularization may not be achieved. Documented global absence of viable tissue may be the only indication to consider heart transplantation over myocardial reperfusion [22].

	Surgical evolution and current techniques

Top Abstract Introduction Physiology Classification Diagnosis Surgical evolution and current... Surgical results Operative risk factors Myocardial scarring and mitral... Mechanical circulatory support Ultrastructural residual and... Conclusions References

 
Surgical correction is the gold standard in the therapy for ALCAPA and has undergone considerable evolution since its inception. One of the earliest operative attempts was by Willis J. Potts from Children’s Memorial Hospital in Chicago, who created an aortopulmonary anastomosis in 2 patients, thereby increasing PA blood flow and hence, left coronary artery oxygen saturation [38]. In 1953, Mustard reported an end-to-end anastomosis between the left common carotid artery and the ALCAPA [39]. Five years later, Case and colleagues [40] discussed the use of PA banding in an attempt to diminish left-to-right shunting through the ALCAPA [40]. In 1959, Sabiston and co-workers [41] successfully performed ligation of the ALCAPA at its pulmonary origin. They convincingly verified the retrograde flow in the left coronary artery and measured a striking increase in left coronary artery pressure when its anomalous origin from the PA was occluded. Cooley and associates [42] reported in 1966, the first creation of a two-coronary system using a saphenous vein graft to the ALCAPA. Two years later, Meyer and colleagues [43] presented the case of a patient who had successful anastomosis of the left subclavian artery to the origin of the ALCAPA.

In 1974, Neches and coauthors [44] were the first to describe the direct reimplantation of the anomalous left coronary artery into the aorta by transferring it with a button of pulmonary artery (Figs 2, and 3). This currently represents the most popular and fully anatomical correction of ALCAPA [3, 5, 19, 23, 45, 46] and is the one we [3, 30, 36] routinely use. Improvisation and creative surgical technique should adapt to anatomical variations to obtain the most harmonious anastomosis; sufficient length, correct angling, and optimal course of the coronary artery to its new origin will make direct reimplantation into the aorta almost always possible [22, 47]. Direct reimplantation of the ALCAPA may be technically more difficult and hazardous in adults because of increased coronary artery friability, diminished vessel elasticity for mobilization, and the potential for tearing and resultant catastrophic bleeding [13, 16]. In these instances, coronary artery bypass grafting with an internal thoracic artery may be more judicious [48, 49].

View larger version (30K): [in this window] [in a new window]   Fig 2. After the second dose of cardioplegia, an opening is created in the left posterolateral wall of the ascending aorta for implantation of the anomalous left coronary button. Care is taken not to injure the aortic valve. This opening is typically approximately one third smaller in size than the button that was created. The large button of coronary artery can then act as a "conduit" for elongation of the left coronary artery. With proper mobilization of the left coronary artery, it is usually quite easy to perform this anastomosis. Once the anastomosis is created (inset), the aortic cross-clamp is removed, and now both right and left coronary arteries are directly perfused (Reprinted from Backer CL, Hillman N, Dodge-Khatami A, Mavroudis C. Anomalous origin of the left coronary artery from the pulmonary artery: successful surgical strategy without assist devices. Semin Thorac Cardiovasc Surg Pediatr Cardiac Surg Annu 2000;3:165–172 [36]; by permission of W. B. Saunders, a Harcourt Health Sciences Co.)

View larger version (26K): [in this window] [in a new window]   Fig 3. The aortic cross-clamp is off. The posterior sinus of the pulmonary artery where the button was harvested is reconstructed with a patch of fresh autologous pericardium. The pulmonary artery is reanastomosed at the site of the transection (inset). This reconstruction of the pulmonary artery with the cross-clamp off helps to minimize the aortic cross-clamp time. In almost all instances, it is possible to perform the entire procedure with two doses of cardioplegia given in the sequence described [36]. (Reprinted from Backer CL, Hillman N, Dodge-Khatami A, Mavroudis C. Anomalous origin of the left coronary artery from the pulmonary artery: successful surgical strategy without assist devices. Semin Thorac Cardiac Surg Pediatr Cardiac Surg Annu 2000;3:165–72 [36]; by permission of W. B. Saunders, a Harcourt Health Sciences Co.)

View larger version (56K): [in this window] [in a new window]   Fig 4. Takeuchi operation: (A) After aortic cross-clamping and cardioplegic arrest, a flap incision in the pulmonary artery is made. (B) Using the pulmonary artery wall flap, an intrapulmonary tunnel is fashioned, running from the anticipated takeoff of the aorta to the orifice of the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). (C) Anastomosis between the intrapulmonary tunnel orifice and a punch opening in the aorta is performed. The Takeuchi repair is completed by patching the defect in the pulmonary artery wall with autologous pericardium or homograft. The arrow shows the newly directed blood flow from the aorta, through the tunnel, and into the ALCAPA orifice.

Administration of cardioplegia for myocardial protection is a crucial element common to all surgical techniques. It is of utmost importance to maintain sufficient filling pressures in the left coronary system and avoid runoff of the cardioplegic solution into the PA. This is achieved most commonly by injecting cardioplegic solution through the aortic root while simultaneously occluding the ALCAPA at its pulmonary origin or by occluding the main pulmonary trunk or PAs individually [22, 24, 36, 54]. Some authors [24] prefer injecting the cardioplegic solution into the pulmonary trunk in a retrograde fashion through the ALCAPA, whereas others [24, 47] use simultaneous administration into both great vessels after they have been cross-clamped. In our experience, the complementary administration of retrograde cardioplegia in repeat doses has been a useful adjunct [36]. To reduce ischemic time, some [24] advocate earlier release of the aortic cross-clamp during reconstruction of the PA.

	Surgical results

Top Abstract Introduction Physiology Classification Diagnosis Surgical evolution and current... Surgical results Operative risk factors Myocardial scarring and mitral... Mechanical circulatory support Ultrastructural residual and... Conclusions References

 
The operative mortality rate for all surgical techniques combined was in the range of 75% to 80% in the early 1980s [21], but it has been lowered to the 0% to 23% range in the current era [3, 5, 19, 22, 31, 36, 46, 54]. After all techniques that reestablish a two-coronary system, a return to normal size of the previously dilated RCA with regression of the intercoronary collateral network has been observed. To date, no difference in long-term LV function or late mortality has been demonstrated between the various surgical techniques that reestablish a two-coronary system (direct aortic reimplantation, left internal thoracic artery graft, left subclavian artery anastomosis, saphenous vein graft and the Takeuchi procedure) [3, 19, 22, 25, 26, 55]. The exception with reduced long-term survival is isolated ALCAPA ligation, which does not result in normalization of LV volume or ejection fraction [3, 45, 56, 57].

Direct reimplantation of the ALCAPA into the aorta is the most frequently adopted surgical technique, and it has gained popularity through increased experience with coronary artery transfer techniques adapted from the arterial switch operation. The reported mortality rate in direct reimplantation ranges between 0% and 16% [19, 22, 24, 31, 36, 46, 47, 54].

In infants, some [45] advocate the Takeuchi operation as the preferred surgical technique. The operative mortality rate of this procedure, (range, 0% to 23%) is comparable to that of direct coronary artery transfer [13, 24, 45, 50, 54, 56]. Complications include varying degrees of supravalvular pulmonary stenosis [13, 31, 54, 58], baffle leaks creating a coronary-PA fistula, and aortic valve insufficiency. Reoperations or catheter interventions are necessary in as many as 30% of patients to correct these complications [45, 54].

Saphenous vein bypass grafting has yielded operative mortality rates of 0% to 38% [3, 13, 14]. Disadvantages of this procedure in infants are the scarcity of vein grafts, both at initial and at redo operations, small vein caliber, and most importantly, late graft occlusion [3]. In a series of 6 adult patients who underwent saphenous vein bypass grafting and direct ALCAPA closure from inside the PA, Moodie and associates [13] reported a graft patency rate of 80% at a mean follow-up of 5.8 years and an increase in mean LV ejection fraction from 0.55 to 0.62 [13].

More recently, use of the left internal thoracic artery has supplanted saphenous vein grafting, but there are few reports of midterm or long-term results [3, 48, 49]. Kitamura and co-workers [48] successfully used bilateral internal thoracic artery grafting to the left anterior descending and circumflex coronary arteries [48]. Extrapolating from the excellent long-term patency rates of internal thoracic arteries for coronary surgical procedures in general [57], Chan and associates [27] recommended it as the procedure of choice in adults with ALCAPA.

Simple ligation of ALPACA at its origin was historically the first definitive surgical correction for this condition [41], with operative mortality rates ranging between 20% and 50% [14, 28, 45] and a late mortality rate of up to 33% [3, 28, 45, 59, 60]. Physiologically, this correction resembles a single coronary artery system and is at greater risk for atherosclerosis [7, 17, 28, 45, 60]. Anecdotal reports of successful simple ligation in older patients exist [45, 61]. Simple ligation of ALCAPA has been performed as a lifesaving emergency procedure [5] and when extensive LV infarction with aneurysm has already occurred [62]. In these circumstances, it has been argued that little is to be gained by additional coronary flow to a nonviable myocardial territory. Sivasubramanian and associates [61] even questioned the necessity of reestablishing a two-coronary system; they claimed that in the absence of electrocardiographic changes or LV dysfunction, simple ligation of the ALCAPA is not unreasonable. However, in favor of establishing a two-coronary artery system, Kececioglu and colleagues [59] demonstrated silent myocardial ischemia by thallium and by stress ECG in patients who had undergone simple ligation. This represents an anatomic substrate for the unacceptably high incidence of sudden death, which should categorically and definitively classify this procedure as a relic of the past. For surviving patients of simple ligation, it is increasingly being recommended that they undergo elective revascularization with bypass grafting, thus reestablishing a two-coronary system [3, 28, 59].

	Operative risk factors

Top Abstract Introduction Physiology Classification Diagnosis Surgical evolution and current... Surgical results Operative risk factors Myocardial scarring and mitral... Mechanical circulatory support Ultrastructural residual and... Conclusions References

 
Various risk factors for operative survival have been identified in the treatment of ALCAPA, including decreased preoperative LV function [20, 22, 26] and young age at operation [26]. Although younger patients are seen with more severe LV failure, surgical intervention results in a more rapid and complete recovery of myocardial function, as assessed by echocardiography [23]. Infants seen later (>10 months) have less depressed LV contractility from increased intercoronary collateralization, but this has not been associated with improved postoperative myocardial recovery [23]. Sauer and associates [26] found extreme RCA dominance (ie, perfusion of the posterolateral LV wall beyond the crux cordis) to correlate positively with operative survival (p < 0.001). As a corollary, they found a left dominant or a balanced coronary circulation to be a risk factor for surgical mortality (p < 0.01). This group wrote that abnormal preoperative ECGs including ST segment elevations in more than two chest leads or in more than one standard lead indicate acute myocardial infarction and correlate with poor operative outcome (p < 0.03).

The severity of preoperative MR has been proposed as a surgical risk factor [58], although this is not supported unanimously [19, 22, 26].

	Myocardial scarring and mitral valve procedures

Top Abstract Introduction Physiology Classification Diagnosis Surgical evolution and current... Surgical results Operative risk factors Myocardial scarring and mitral... Mechanical circulatory support Ultrastructural residual and... Conclusions References

 
The surgical correction of ALCAPA strives to address not only the coronary problem, but also the other end points of ischemic insult to the left heart. Left ventricular free wall aneurysm and mitral valve insufficiency are the two potential surgical targets, and treatment of the latter is still controversial.

Most myocardial scarring does not result in the formation of an aneurysm, and postinfarction ventricular septal defects or cardiac rupture [63] are extremely rare in patients with ALCAPA. Ischemic yet viable myocardium, as assessed by stress thallium 201 or dipyridamole stress echocardiography, will recover postoperatively after reperfusion by any procedure creating a two-coronary system [22, 24, 25, 31, 45, 54, 56, 58]. This explains the substantial improvement in LV function within days after operation [19, 23], best illustrated by the possibility to wean from mechanical circulatory support, even in cases of extreme preoperative myocardial compromise [31]. As a corollary, during initial surgical intervention for coronary artery revascularization, concomitant resection of LV muscle is not justified [22], although anecdotally advocated by some [62] or performed in grave situations after cardiac rupture [63].

Mitral regurgitation is related both to ischemic LV dilatation with mitral annulus enlargement and to ischemic dysfunction of the papillary muscles [64, 65]. We [36] and others [22, 24, 31, 46, 47] agree not to address moderate or even severe mitral insufficiency by repair or replacement at the initial operation, the primary goal of which is coronary reperfusion and myocardial salvage. In the setting of severely compromised ventricular function, the added ischemic time to perform mitral valve procedures is potentially more deleterious than helpful [31, 46]. This policy is further supported by the relatively disappointing results from surgical treatment of ischemic MR in adults [65]. In ALCAPA, even severe mitral insufficiency has been reported to fully regress after reperfusion alone in the majority of cases [23, 31], and immediate postoperative mild to moderate MR is acceptable [22, 24, 47]. After improvement in LV function, persistent symptomatic or hemodynamically significant MR can always be treated at a later time [3, 22, 25, 26, 46].

Some authors [54, 66], however, recommend routine mitral valve repair at the time of coronary artery revascularization on the grounds that early postoperative cardiac output is improved with a resultant decrease in morbidity. It remains speculative whether their excellent results would have been achieved without associated mitral valve procedures. Others advocate mitral annuloplasty [19, 24, 64] or mitral valve replacement [45, 64] only in the presence of severe MR. Recently, Huddleston and associates [46] emphasized the importance of persisting, recurring, or worsening MR after coronary reperfusion without associated mitral valve surgical procedures. At coronary angiography, they found severe stenosis of the reimplanted ALCAPA in 2 patients with worsening cardiac function and mitral insufficiency, who underwent reoperation for coronary artery revision and mitral valve repair. In patients with persisting or recurring MR, these authors stressed the importance of documenting coronary patency and recommended cardiac catheterization prior to reoperation for a mitral valve procedure.

	Mechanical circulatory support

Top Abstract Introduction Physiology Classification Diagnosis Surgical evolution and current... Surgical results Operative risk factors Myocardial scarring and mitral... Mechanical circulatory support Ultrastructural residual and... Conclusions References

 
Encouraging results have been achieved with the use of cardiac mechanical support as a bridge to recovery in the postoperative setting of ALCAPA [8, 24, 31, 46, 67–72]. Such support measures include LV assist devices [8, 31, 68–70] extracorporeal membrane oxygenation [24, 46, 71], and intraaortic balloon pumps [72] in older children. In a report by del Nido and associates, [8], 5 survivors among 7 infants who underwent surgical correction of ALCAPA and intractable LV failure were successfully supported with an LV assist device. When intractable ventricular arrhythmias are an ongoing postoperative problem despite successful coronary reperfusion, an LV assist device may not be applicable, and total cardiac support with extracorporeal membrane oxygenation should be strongly considered [8]. In a series of 16 infants with ALCAPA undergoing direct aortic reimplantation, our group [36] encountered no mortality and no requirement of postoperative mechanical support. We speculate that a strategy using both antegrade and retrograde blood cardioplegia, combined with appropriate postoperative inotropic support may allow for favorable results without the need of mechanical circulatory support. However, as the degree of preoperative patient instability and myocardial impairment varies greatly, the full array of circulatory support should always be readily available if required. Given the excellent results that maximally enhance patient survival, LV assist devices and extracorporeal membrane oxygenation play an obvious integral part in the modern surgical treatment of patients with ALCAPA.

	Ultrastructural residual and follow-up modalities

Top Abstract Introduction Physiology Classification Diagnosis Surgical evolution and current... Surgical results Operative risk factors Myocardial scarring and mitral... Mechanical circulatory support Ultrastructural residual and... Conclusions References

 
Although improvement in myocardial function is expected after surgical correction of ALCAPA [22, 23, 56, 73], normalization of LV function may take as long as 22 months [22, 23, 31, 56]. This slow recovery results from preoperative ultrastructural abnormalities caused by chronic myocardial hypoperfusion at the cellular level, which need time to normalize [18, 22, 56, 74]. More rapid regression of LV enlargement and more complete recovery of LV function constitute the rationale to perform earlier surgical intervention to reduce the duration of the initial ischemic insult [23]. Without reperfusion, cellular structural adaptation progresses and contributes to further LV dilatation and worsening congestive heart failure [58]. In an intraoperative study of subepicardial and subendocardial biopsy specimens from 5 patients with ALCAPA, Shivalkar and associates [74] found an important degree of fibrosis with altered but viable myocytes. Similarly, Smith and coauthors [18] reported that 14 heart specimens from unoperated patients with ALCAPA who did not have operation, showed endocardial and subendocardial fibrosis along with patchy myocardial necrosis in regions of dilatation and aneurysm of the left ventricle. In patients with MR, papillary muscle damage and leaflet dysplasia were present. Schwartz and colleagues [58] explained the postoperative delay in LV recovery (1 to 7 months) as the time needed for myocyte hyperplasia and compensating hypertrophy of remaining viable myocytes.

These ultrastructural studies demonstrate the complexity of assessing abnormal hibernating myocardium and distinguishing it from necrotic tissue in patients with ALCAPA, both preoperatively for diagnostic purposes and postoperatively for follow-up [37]. Various studies with thallium 201 scanning with dipyridamole or adenosine-induced hyperemia show the limitations of detecting ischemic myocardium [55, 56, 75]. Lambert and associates [19] followed half of their symptom-free patients with echocardiography and a stress thallium test at a median period of 4.9 years postoperatively. On echocardiography at rest, 86% had a normal shortening fraction. With stress testing, however, many patients showed transient defects, and some showed persistent defects with incomplete reperfusion in the anteroseptal and anterolateral segments of the left ventricle. Despite good function, persistent LV enlargement was noted in 73% of the patients.

Using adenosine-induced hyperemia on positron emission tomographic scan and nitrogen 13-labeled ammonia, Singh and colleagues [75] compared left coronary artery-dependent segments with a baseline RCA-dependent territory in 11 asymptomatic patients at a mean follow-up of 16 years after operation for ALCAPA. The resting ejection fraction was 63 ± 6, but with adenosine, 10 of the 11 patients demonstrated perfusion defects in the anterior and anterolateral walls compared with the RCA-perfused territory. Despite a maximum exercise test in 9 of the 11 patients, there was chronotropic impairment in 3 patients (<85% of predicted heart rate), blunted blood pressure in 4 (<30 mm Hg increase in systolic blood pressure), and ST segment depression in 2 patients. The authors concluded that myocardial flow reserve is significantly reduced in left coronary artery-dependent territories (p < 0.001) and that the anaerobic threshold is lower than in the control RCA-dependent territory. An increase in collagen tissue surrounding viable myocytes with patchy fibrosis of the interstitial tissue was documented [75]. Singh and co-workers postulated these structural abnormalities were responsible for the diminished vasodilator response of the coronary microcirculation in response to adenosine-induced exercise. In the same study [75], on the basis of angiographic evidence of graft status (patent versus occluded), the authors recommended close follow-up and anti-ischemic medication (ß blockers), even in patients with angiographically normal grafts. Paridon and associates [55] studied 11 asymptomatic patients after operation for ALCAPA; they had no respiratory limitation to exercise and achieved maximum aerobic effort. Abnormal ECGs with ST segment depression and ventricular arrhythmias were found in 7 of the 11 patients, whereas only 4 had an abnormal result with stress thallium scintigraphy. Chronotropic impairment was present, and exercise and reperfusion scanning showed residual ischemia that a regular thallium test would have missed. As with patients after the arterial switch procedure, coronary angiography may be the only definitive diagnostic test to assess left coronary artery patency [3, 46], although the timing of such an invasive procedure remains to be defined in asymptomatic patients.

	Conclusions

Top Abstract Introduction Physiology Classification Diagnosis Surgical evolution and current... Surgical results Operative risk factors Myocardial scarring and mitral... Mechanical circulatory support Ultrastructural residual and... Conclusions References

 
A high index of suspicion and appropriate diagnostic modalities should allow rapid diagnosis of ALCAPA in any patient seen with dilated congestive cardiac failure. The surgical results of operations that reestablish a two- coronary artery circulation have dramatically improved in recent years in this otherwise fatal disease. Regardless of the degree of preoperative ventricular impairment, an aggressive early surgical approach is warranted, as it represents the only possibility to salvage hibernating but viable myocardium [7, 8, 22, 24, 36]. Perioperative mechanical support with LV assist devices and extracorporeal membrane oxygenation has improved survival in patients with ALCAPA. They constitute invaluable new adjuncts for keeping patients alive until or after surgical correction [8, 24, 31, 67, 69–72] and should preclude the need of cardiac transplantation. Documented massive infarction may be the only justification for cardiac transplantation, although this situation is disappearing. Regardless of the degree of insufficiency, we do not address the mitral valve at the initial surgical intervention, a view shared by most surgeons [22, 24, 31, 46, 47] though it is not unanimous [19, 45, 54, 66]. Residual or recurrent MR is present in up to one third of patients and can be dealt with according to its degree of severity. Also, it should raise the suspicion of coronary patency, which needs to be confirmed by cardiac catheterization [46]. Although postoperative improvement in LV function is the rule, some degree of chronic impairment from preoperative structural abnormalities may persist.

The best follow-up method in patients with ALCAPA is unknown, as ECG, Holter monitoring, and stress thallium scanning have shown equivocal results. Most often, these underscore any degree of ongoing or recurring LV dysfunction, which may be apparent only under extreme conditions of stress testing. Cardiac catheterization with coronary angiography will assess repair patency, but its use and timing in asymptomatic patients need to be defined. Close long-term follow-up of these patients is necessary to better understand the "corrected natural history," which is the current expected goal after surgical treatment of ALCAPA.

	References

Top Abstract Introduction Physiology Classification Diagnosis Surgical evolution and current... Surgical results Operative risk factors Myocardial scarring and mitral... Mechanical circulatory support Ultrastructural residual and... Conclusions References

 

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