Identification of patients at risk is challenging [ 6 , 53 - 55 ]. While fetal or neonatal presentation of Ebstein anomaly is associated with a poor outcome [ 39 , 56 ], adults have a much better prognosis [ 52 , 57 ]. Surprisingly, supraventricular arrhythmia does not appear to be associated with worse outcome [ 39 , 57 , 58 ]. There are no randomized trials comparing surgical and non-surgical management of Ebstein anomaly. Atrial arrhythmia is the most frequent early complication.
Survival and functional status did not differ significantly between groups undergoing TV repair or replacement [ 50 , 59 ]. Most patients with CTGA are now diagnosed in infancy or childhood. Some without associated defects go undetected until the 3rd to 6 th decades and rare patients survive a lifetime with no symptoms [ 60 , 61 ].
Consequently, the population followed in most adult congenital heart disease clinics is a mix of operated and unoperated patients. There are two types of CTGA. In the most common type, the atria and the abdominal organs are in the usual locations situs solitus. Further, the great arteries are transposed so that the left-sided aorta is aligned with the RV and the right-sided pulmonary artery with the LV.
Systemic venous blood reaches the pulmonary artery through the LV and pulmonary venous blood the aorta through the RV [ 62 ]. A much less common form of CTGA occurs in situs inversus and is the mirror image of the type described above. In this type, there is inversion or left-right mirror imagery of the atria and abdominal organs. Here the systemic venous blood returns to the left-sided RA, left-sided LV and left-sided and posterior pulmonary artery.
Pulmonary venous blood returns to the right-sided LA, right-sided RV and rightsided and anterior aorta. Often there is also an additional posterior AVN, but it is usually hypoplastic and rarely connects to a penetrating bundle [ 66 ]. This anterior location of the AVN and penetrating bundle makes them vulnerable to complete heart block [ 6 ].
The bundle branches are inverted along with the ventricles. Because of the anterior location of the node and penetrating bundle, the conduction system passes in the superior rim of a VSD [ 66 ]. The coronary anatomy is quite different from the normal heart. The accompanying article by Baraona et al. In the absence of associated defects, the physiology in CTGA is normal. A VSD plus pulmonary stenosis can result in a right-to-left shunt and cyanosis like tetralogy of Fallot or a balanced circulation with only a small shunt in one direction or the other.
Isolated pulmonary stenosis causes pressure overload of the LV and if severe can cause failure of the pulmonary ventricle. The systemic RV fails sooner with associated defects and later without Figure Without associated defects, CTGA patients can be asymptomatic until the third to sixth decades [ 60 , 61 ]. Typical presenting symptoms include a murmur, cyanosis, bradycardia, heart failure, and arrhythmia [ 68 ]. In some cases the diagnosis is made incidentally by cardiac testing for other reasons.
Physical exam reveals a single, loud S2 due to the anterior location of the aorta [ 62 ] and difficulty hearing the soft closure sound of the posterior pulmonary artery [ 68 ]. A holosystolic murmur at the left sternal border, often with a thrill, indicates a VSD [ 69 ]. Pulmonary stenosis causes a systolic ejection murmur at the left or right upper sternal border [ 70 ].
Cyanosis and clubbing could be due to pulmonary stenosis with VSD tetralogy of Fallot physiology or to Eisenmenger syndrome [ 13 ]. A holosystolic murmur at the apex is due to TR [ 13 ]. Q waves in the inferior ECG leads are due to right-to-left septal depolarization and can be misinterpreted as a prior inferior myocardial infarction [ 70 ].
About one-half of patients have 1st degree AV block and the risk of 3rd degree AV block increases with age [ 69 , 70 ]. The P wave is negative in lead I in CTGA in situ sinversus because the atria are inverted; however, ventricular septal activation is normal because the ventricles are normally located [ 62 ]. Signs on the chest radiograph suggestive of CTGA are a prominent left-sided ascending aorta [ 62 ] and dextrocardia [ 70 ].
Two-dimensional echocardiography is diagnostic [ 62 , 71 ]. CMR is the standard for assessment of ventricular size and function, and can be useful to define anatomy if echocardiographic windows are poor Figure 19 [ 13 ]. Extended ECG monitoring is used to diagnose arrhythmias and to estimate average and slowest heart rate in patients with heart block.
Exercise testing provides an objective assessment of functional capacity. Cardiac catheterization can be useful to obtain physiological information or if non-invasive testing is not diagnostic [ 13 ]. Medical therapy for CTGA is useful to control arrhythmias [ 13 ] and to treat heart failure. The optimal medical strategies for treatment of systemic RV dysfunction are unknown.
Diuretic therapy is effective for management of fluid balance. It is unclear if renin-angiotensin activation plays a role in systemic RV dysfunction [ 73 ]. On the other hand, a small pilot study of carvedilol in CTGA patients with RV dysfunction showed positive remodeling and increased exercise duration [ 74 ]. Invasive electrophysiology treatment is indicated for atrial tachyarrhythmias catheter ablation and for bradycardia due to heart block pacemaker therapy.
Single site pacing can induce dyssynchrony and rightward septal shift can worsen TR [ 13 ]. Biventricular pacing in CTGA is difficult due to the unusual cardiac venous anatomy [ 75 ]. In adolescent and adult patients, surgical therapy is usually directed to a specific hemodynamic abnormality. Patients with severe or progressive TR are candidates for TV replacement [ 13 ]. Timing of surgery should avoid deterioration of RV function [ 76 ]. Closure of a VSD should be considered if the RV is dilated and pulmonary vascular resistance is not significantly elevated.
Severe aortic regurgitation likely from progressive root dilation with RV dilation should be addressed before RV function deteriorates. Relief of isolated pulmonary stenosis has been advocated, but it is not clear that this is beneficial unless LV pressure is suprasystemic. Some highly select adult patients with CTGA are candidates for an anatomic correction. The success of anatomic repair is dependent on the LV being adequately prepared to generate systemic blood pressure [ 69 ].
Included are patients with severe, but remediable, pulmonary stenosis and those with a large outlet VSD. The procedure switches both venous inflow Mustard and arterial outflow arterial switch or Rastelli operation, that is baffling the LV to the aorta through the VSD and placement of a RV to pulmonary artery conduit [ 13 ]. Adult patients with symptomatic RV failure are candidates for heart transplantation because re-training the LV to perform systemic work after years of functioning as the pulmonary ventricle has been mostly unsuccessful [ 78 ].
Previously operated CTGA patients might also undergo surgery for various reasons including replacement of a dysfunctional LV- or RV-to-pulmonary artery conduit and aortic valve surgery for aortic regurgitation [ 13 ]. TR begins in the second decade, becomes moderate or more in the third decade, and increases in severity and prevalence thereafter. Progressive TR begets more dilation of the systemic RV, which in turn contributes to more regurgitation [ 70 ]. Congestive heart failure with pulmonary edema often ensues between the fourth and sixth decades [ 60 ].
Aortic regurgitation, not previously reported in CTGA, is now known to be a frequent problem in this population [ 69 ]. Outcomes for conventional repair have improved from lessons learned over the past three decades. The outcomes for various cohorts are shown in Table 2 [ 79 - 83 ]. Infants with DTGA present with cyanosis in the first days of life. Consequently, patients presenting to an ACHD center have had corrective surgery in infancy or childhood. The primary abnormality in DTGA is ventriculo-arterial discordance.
The atria and ventricles are normally located but the great arteries are aligned with the incorrect ventricle, that is, the aorta is aligned with the RV and the pulmonary artery with the LV [ 62 ]. The aorta is usually anterior and rightward of the pulmonary artery but the great arteries can be side-by-side and rarely the aorta is posterior or anterior and leftward [ 6 ]. Coronary artery anatomy is variable and important for the arterial switch operation ASO [ 85 ].
The article by Baraona et al. Patients born before the early s most likely underwent an atrial switch operation Mustard [ 86 ] or Senning [ 87 ] or a Rastelli [ 88 ] operation. Patients born after the late s were most likely repaired using the ASO Figure After an atrial switch operation the ventricular and arterial anatomy are unchanged. A baffle is placed in the atria to redirect systemic venous blood to the MV and pulmonary venous blood to the TV Figures 20, In contrast, after the ASO [ 89 ] the great arteries are inverted so that the aorta connects with the posterior root previously pulmonary root and the pulmonary artery with the anterior root Figures 20, The aorta is pulled through between the branch pulmonary arteries allowing the branches to straddle the aorta.
The coronary arteries are translocated from the anterior to the posterior root Figure A Rastelli operation has been used in patients with a large VSD and severe pulmonary stenosis Figure Here the LV is baffled to the aorta through the VSD, the pulmonary trunk is closed, and a conduit is placed from the RV to the distal pulmonary artery [ 88 ].
There are alternative operations that accomplish the same type of repair and might have advantages in specific cases [ 90 ]. Prior to corrective surgery, the systemic and pulmonary circulations are in parallel instead of in series. Consequently, desaturated blood from the systemic veins is returned to the body and saturated blood from the pulmonary veins is returned to the lungs. At repair, the circulations are placed in series either by switching the inflow sources atrial switch operation or by switching the outflows arterial switch operation and Rastelli.
In addition to routine care, patients after atrial switch operation might present with fatigue, arrhythmia, venous congestion, or symptoms of heart failure. Patients are also referred during pregnancy for more intensive surveillance. Subpulmonary stenosis, a frequent finding, is indicated by an ejection murmur higher up the left sternal border.
Venous congestion or hepatomegaly could signal systemic venous pathway obstruction or heart failure. Patients who have had an ASO are usually asymptomatic but might complain of chest pain or exercise limitation. The physical exam is usually unremarkable but a pulmonary outflow murmur or a diastolic murmur of pulmonary or aortic insufficiency might be audible.
Various rhythm abnormalities might be evident including bradycardia, junctional rhythm, or complete heart block [ 13 ]. The chest radiograph often shows cardiac enlargement. The echocardiogram might not demonstrate the atrial baffle and venous pathways adequately after an atrial switch operation. CMR is excellent for anatomic evaluation, measurement of ventricular size and function and myocardial characterization Figures 24, CT angiography can be used for functional assessment in patients with an implantable device such as a permanent pacemaker or automated defibrillator and is excellent for coronary artery anatomy [ 91 ].
Extended ECG monitoring is useful to detect and diagnose arrhythmias. Exercise testing is an excellent method to follow functional capacity or detect ischemia from coronary artery abnormalities. After an ASO the echocardiogram shows the anterior pulmonary artery paralleling the aorta and the branch pulmonary arteries passing posteriorly on either side of the ascending aorta.
CMR demonstrates overall anatomy, coronary artery anatomy, and ventricular size and function Figure CT angiography is also useful for assessment of the coronary arteries. CMR provides anatomic evaluation as well as functional assessment after the Rastelli operation Figures 27, Sinus node dysfunction is the most frequent complication of an atrial switch operation. Tachy-brady syndrome is common in this setting as is atrial flutter.
Atrial tachyarrhythmias during the operative period, permanent heart block, and small size at surgery are independent risk factors for sudden death [ 92 ]. Risk factors are unclear, but excess hypertrophy is likely to be important [ 6 , 95 ]. Recurrent ischemia contributes to ventricular dysfunction as evidenced by regional wall motion abnormalities, perfusion defects and late gadolinium enhancement documented decades after a Mustard procedure [ 96 ].
TR usually accompanies RV dysfunction and can contribute to further deterioration [ 93 ]. Other late complications include venous pathway obstruction and baffle leaks Figures 24, The SVC pathway is most often involved and usually decompresses via the azygos vein so that adults are rarely symptomatic [ 97 ].
It is usually discovered during placement of a transvenous pacemaker for sinus node dysfunction. Inferior vena cava pathway obstruction is rare [ 13 ]. Baffle leaks are more frequent but usually small. These persistent communications can be a source of paradoxical embolization especially if a transvenous pacemaker is in place [ 97 ].
Obstruction of the pulmonary venous pathway Figure 25 is more likely with the Senning operation but is rare late after surgery [ 97 ]. Pulmonary venous pathway obstruction is another cause of elevated pulmonary artery pressure [ 98 ]. Complications are mainly conduit obstruction and subaortic stenosis from inadequate enlargement of the VSD [ 99 ]. LV dysfunction has been described mostly in the setting of severe subaortic stenosis. There is a small but persistent incidence of sudden death, presumed to be arrhythmic in nature [ 99 ].
Description
Pulmonary artery stenosis is the most frequent complication following the ASO. Mechanisms include inadequate growth of the suture line, scarring and retraction of the material used to fill the coronary artery button sites, and tension at the anastomotic site if there is inadequate mobilization of the distal pulmonary arteries [ ].
Arrhythmia is infrequent and sudden death is rare [ 6 ]. The LV in the systemic position maintains good function over time. There is a modest risk for neo-aortic valve regurgitation related in part to neo-aortic root dilatation. Patients with a VSD tend to be at higher risk for neo-aortic valve regurgitation [ ]. Although pulmonary hypertension after the ASO is infrequent, it was a cause of late death in one study [ ].
Arrhythmia management is the most common treatment. Medical or transcatheter treatment of atrial tachyarrhythmia can be challenging. Sinus node dysfunction with tachybrady syndrome is an indication for pacemaker therapy. SVC pathway obstruction can be treated by stent placement, but it is important to define the coronary artery anatomy before undertaking an interventional procedure at the base of the heart [ 67 ].
A baffle leak can often be closed percutaneously with a device. Heart failure symptoms are usually treated with an ACE inhibitor and beta blockade but there is little evidence of efficacy []. Diuretic therapy can be used to maintain fluid balance. Heart transplantation should be considered for end-stage heart failure in atrial switch patients. Replacement of the RV-PA conduit or homograft is the most frequent re-operation after the Rastelli operation.
Enlargement of the VSD to alleviate subaortic stenosis is a less frequent secondary procedure. Stent placement is usually effective for branch pulmonary artery stenosis. Dense scarring of the proximal outflow renders it less amenable to percutaneous therapy and usually requires re-operation. The late survival is higher for the ASO compared with the atrial switch procedures, with fewer long-term complications [ ].
TOF results from leftward and superior displacement of the infundibular or outlet septum, apparently related to abnormal rotation of the outflow during embryogenesis. In extreme cases the RV outflow and even the pulmonary trunk are atretic [ 62 ]. The accompanying article in this issue by Baraona et al.
Patients presenting to an ACHD center have undergone repair in infancy or childhood. Patch augmentation of the RV outflow, division or resection of obstructing muscle, and bypass of obstruction using a RV-pulmonary artery conduit are typical approaches to repair. Until the last decades wide patch augmentation of the outflow was standard treatment to avoid any residual stenosis, at the expense of creation of free pulmonary regurgitation PR Figure More recently, the emphasis has shifted to preservation of PV function as the late deleterious effects of chronic PR have become apparent.
Patients repaired in the last 2 decades are likely to have had a transatrialtranspulmonary approach with limited or no infundubulotomy.
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Efforts are now made to avoid a trans-annular patch [ 62 ] or to place the patch in such a way as to preserve valve function as much as possible. In patients with pulmonary atresia or with a major coronary artery crossing the RVOT, an RV-to-pulmonary artery conduit is used Figure Additional defects such as ASD2 are closed at the time of complete repair and any previously constructed shunt Figure 31 taken down [ 6 ]. The physiology of TOF depends on the severity of outflow obstruction and can vary from absence of cyanosis pink tetralogy to ductus arteriosus-dependent pulmonary circulation in cases with pulmonary atresia.
After repair the majority of patients have normal oxygen saturation and no residual shunt. The predominant physiology is RV volume overload from moderate or more PR. Few patients have residual pulmonary stenosis. RV dysfunction and failure as a consequence of chronic volume overload are seen late after repair. LV dysfunction is also seen late after repair, even in the absence of a residual VSD or other volume overload [ ]. The mechanism remains unclear.
The unoperated adult with TOF is rarely encountered in developed countries but is less rare where access to health care is more limited. Cyanotic and clubbed, these patients present with exercise limitation, secondary erythrocytosis, stroke or heart failure.
S2 is usually single and patients with a patent RV outflow have a systolic ejection murmur in the pulmonary area while those with pulmonary atresia have continuous murmurs over the chest and back due to aorto-pulmonary collaterals [ 13 ]. Most patients remain asymptomatic for years after repair of TOF. Some complain of exercise limitation, arrhythmia, or heart failure symptoms.
Physical exam of the chest provides clues to the treatment history. A lateral thoracotomy scar indicates initial palliation with an aorto-pulmonary shunt. An absent or weak radial pulse on the side of the thoracotomy suggests a classical Blalock-Taussig shunt. A sternotomy scar usually indicates complete repair. S2 is single because the PV has been damaged or even removed at repair. There is often a low-to-medium pitched systolic ejection murmur at the left sternal border due to residual pulmonary stenosis.
A diastolic decrescendo murmur in the same area indicates PR. There might be concomitant aortic regurgitation, resulting from aortic root dilation. An aortic regurgitation murmur is high-pitched in contrast to the low-pitched PR murmur. A residual VSD is indicated by a holosystolic murmur at the lower sternal border. Patients who have undergone only palliation with an aorto-pulmonary shunt have a continuous murmur Figure Right bundle branch block is seen routinely on ECG, especially following a trans-ventricular approach to repair common before Atrial fibrillation or flutter might also be present and is more common with increasing age [ ].
The chest radiograph often shows a large heart due to RV enlargement. Echocardiography is used in the routine evaluation of repaired TOF patients [ 3 ]. CMR has emerged as the modality of choice for evaluating ventricular size and function. In addition, the anatomy of the RV outflow Figure 32 , pulmonary arteries, aorta and aorto-pulmonary collaterals can be seen in detail, which is necessary for surgical planning.
The severity of PR can be quantified Figure 33 , as can any residual shunt. Lastly, myocardial viability can be assessed using late gadolinium enhancement Figure 34 [ ]. If there are contraindications to CMR, CT is also excellent for evaluation of TOF [ 13 ], however, this procedure exposes the patient to ionizing radiation. The most common late complication is chronic PR. Residual RV outflow obstruction and branch pulmonary artery stenosis Figure 35 [ 13 ] are less frequent but important late complications. Conduit dysfunction is expected and virtually all conduits undergo replacement or interventional treatment at some point.
TR can be progressive and compounds the RV dilation. Late RV dilation and dysfunction are common [ ]. Arrhythmias are another important late complication. Prevalence of ventricular arrhythmias increases with age and is associated with LV dysfunction [ ].
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Patients who present with arrhythmias should undergo evaluation for a hemodynamic cause [ 13 ]. LV dysfunction late after repair of TOF is likely due to adverse ventricular-ventricular interaction with the dilated, often dysfunctional RV and appears to be at least as significant a risk factor for arrhythmia and sudden death as RV dysfunction [ ]. PV replacement is the treatment for chronic PR Figure 36 but the timing and method remain key management questions. PV replacement is usually recommended for patients with severe PR and symptoms or decreased exercise performance.
Replacement is also reasonable for patients with severe PR and RV dysfunction, or with symptomatic or sustained atrial or ventricular arrhythmias. Surgical valve replacement is the only option currently available for the native RV outflow tract. Operative mortality for PV replacement is low [ ] and exercise performance improves [ ]. Although RV volume decreases it often does not normalize, especially if the RV was severely dilated preoperatively [ , ].
While the question of operating too late has been raised [ ], performing PV replacement too early is also an issue, as all prosthetic valves have a limited life span. RV outflow reconstruction or RV remodeling surgery [ ] might be considered in conjunction with PV replacement. Abnormally contracting segments can stem from infundibular resection or perioperative ischemic insult [ ]. Tricuspid valve annuloplasty can also be performed for moderate or severe TR [ 6 ]. Treatment options for failure of a RV-to-pulmonary artery conduit include replacement of the conduit, stent implantation to treat stenosis, and percutaneous valve implantation to treat regurgitation [ ].
Late aortic root dilation is common [ ] and might require intervention, although the indications are not clear. Balloon dilation or stenting should be considered for branch PA stenosis if flow in the artery is reduced and especially when accompanied by PR [ 6 ]. Atrial arrhythmias can be addressed by a Maze procedure at the time of PV replacement [ 6 ]. Patients with documented sustained VT or aborted sudden cardiac death should receive an ICD for secondary prevention [ ].
While there remains no consensus on the management of non-sustained VT found on surveillance monitoring [ 13 ], programmed ventricular stimulation can be of use in risk stratifying the asymptomatic patient. Survival after repair of TOF is less than expected for the general population at all times and the rate of attrition increases sharply 25 years after surgery Figure 37 [ ].
The similarity of the survival curve shown in Figure 37 to that for congenital PR [ ] strongly suggests a role for chronic RV volume overload in late mortality. Sudden death due to ventricular arrhythmia is the most common cause of death after surgical repair of TOF [ ]. Older age at complete repair, a prior Waterston or Potts shunt, placement of an outflow tract patch, and earlier year of surgery were found to be risk factors for late mortality in patients operated during the ss [ , ]. Peripheral pulmonary stenosis and TR were also associated with ventricular tachycardia [ ].
As a result of these late complications, current surgical approaches focus on preserving the PV annulus, accompanied by aggressive resection of RVOT obstruction [ ].
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Only short to mid-terms results are available but this approach seems to produce less RV dilation [ ]. The prognosis without surgical intervention in childhood is poor, although rare patients with well-balanced circulations survive into adulthood with reasonable functional capacity [ ]. Long-term palliation involves several staged procedures leading to the Fontan circulation. Congenital heart defects typically staged toward this type of palliation include tricuspid atresia, double-inlet LV, hypoplastic left heart syndrome, hypoplastic right heart syndrome, and other more rare defects.
Clinical use of complete right heart bypass was first reported by Fontan and Baudet in as palliation for tricuspid atresia [ ]. Since then, several modifications of the original procedure have been devised, always with the goal of separating the circulations by directing systemic venous return to the pulmonary arteries without an interposed ventricle, and using the one functional ventricle in the systemic circulation. The evolution of the Fontan operation over the last 40 years has been reviewed recently [ ]. Virtually all adults with functionally one ventricle followed in adult congenital heart disease centers have undergone some variant of the Fontan operation Figure Most patients operated before the s had connection of the RA to the pulmonary arteries, either directly or by means of a conduit, and closure of any communication between the RA and the systemic circulation Figure More recent modifications include the lateral tunnel connection [ , ] and the extracardiac conduit connection [ , ].
These modifications have superior flow characteristics and little or none of the RA is exposed to high venous pressure [ ]. The extracardiac conduit also avoids extensive suture lines in the RA. The benefits of these modifications include better hemodynamics and less potential for arrhythmias [ , ]. An atrial septal defect or fenestration is often created in the baffle to reduce systemic venous pressure and increase preload to the systemic ventricle Figure 38B [ ]. Although the fenestration allows a small right-to-left shunt and causes mild arterial desaturation, it increases cardiac output early postoperatively and reduces the duration of pleural drainage following surgery [ , ].
The fenestration can usually be closed percutaneously later in life to eliminate residual cyanosis [ ]. Pulmonary blood flow is dependent on systemic venous pressure, a measure of the residual energy imparted to the blood by the systemic ventricle. To maintain cardiac output, the mean systemic venous pressure must approximate the mean pulmonary artery pressure in the normal circulation. This means that systemic venous pressure is chronically elevated at least mm Hg. Further, the capacity to increase systemic venous pressure, and subsequently pulmonary blood flow, is quite limited.
Systemic ventricular preload is determined by the pulmonary blood flow. At rest the systemic ventricle is preload deprived because the pulmonary blood flow is limiting. Under these circumstances preload is the major determinant of cardiac output [ ]. Modest changes in inotropy and heart rate have little effect on cardiac output. After load reduction also has little effect on cardiac output but can cause hypotension because of the limitations imposed by preload deprivation. During exercise, pulmonary blood flow increases approximately linearly with mean systemic venous pressure.
Consequently, doubling cardiac output requires a mean venous pressure of mmHg, which is sustainable only for short periods of time. Chronic preload deprivation appears to have deleterious effects on systemic ventricular diastolic and systolic function and might explain the relentless deterioration of function observed in these patients.
In summary, cardiac output is dependent on systemic ventricular preload while inotropic state, heart rate and after load have minor effects except at extremes. Preload is a function of pulmonary blood flow which is determined by total pulmonary resistance pulmonary vascular resistance plus any gradient in the Fontan pathway , probably the most important determinant of functionality of the Fontan circulation. Adult Fontan patients are followed at regular intervals in adult congenital heart centers. Symptoms might include limited functional capacity, arrhythmia, edema, serous effusions, heart failure symptoms, diarrhea, and stroke.
Physical exam findings include prominent veins, hepatomegaly, and edema. S2 is single because there is only a systemic arterial circulation. A stenotic murmur indicates systemic outflow obstruction while a holosystolic murmur means AV valve regurgitation or rarely a communication between the ventricle and the pulmonary circulation. A continuous murmur is usually due to a persistent aortopulmonary connection such as a shunt or collateral vessel.
The echocardiogram is used routinely for follow-up but it has limitations. Useful for valvar and ventricular function and for detection of outflow obstruction, echo often produces poor images of the Fontan pathway, has a poor sensitivity for detecting thrombus in the venous pathway, and generally cannot image the branch pulmonary arteries.
Conversely, CMR is excellent for anatomic and functional evaluation Figures 40, 41 and fair for detection of thrombus. Extended ECG monitoring is essential for detection and diagnosis of arrhythmia. Monitoring of blood chemistry is important to detect renal or hepatic dysfunction. Adult patients with Fontan physiology are at risk for multiple complications. Those with an atriopulmonary connection are at particularly high risk for RA dilation with thrombus formation and atrial arrhythmias [ ].
Both problems lead to diminished cardiac output, reduced exercise capacity and diminished quality of life. The prevalence of thrombosis in the venous pathway is unknown. Systemic ventricular outflow obstruction causes hypertrophy and increased ventricular end-diastolic pressure. Examples include subaortic stenosis due to restriction of the VSD or bulboventricular foramen in patients with double-inlet LV or recurrent aortic coarctation in those with hypoplastic left heart syndrome. Abnormal AV valve morphology puts patients at risk for valvar regurgitation.
Chronic AV valve regurgitation is associated with increased Fontan pathway pressure, diminished ventricular compliance, systolic and diastolic dysfunction, as well as significant arrhythmias [ ]. Other less common complications include protein-losing enteropathy, plastic bronchitis, liver dysfunction, and stroke.
Closure of a fenestration or other interatrial communication has been undertaken to treat cyanosis or systemic embolization, usually by percutaneous device placement [ 10 ]. Cardiac output tends to decrease following closure of the fenestration likely due to reduction of systemic ventricular preload [ ]. Obstruction of the Fontan pathway or of the pulmonary arteries can be treated by stent placement or by surgery.
The Fontan conversion was proposed in the s to improve outcomes of adult survivors of atriopulmonary connection [ ]. This procedure involves revision of the original Fontan pathway to an extracardiac conduit, with branch pulmonary artery reconstruction and RA reduction when necessary. This is accompanied by a Maze type of arrhythmia surgery and placement of a permanent pacemaker [ ].
Residual associated anatomical lesions such as systemic outflow obstruction or AV valve regurgitation can be addressed at the time of surgery [ ]. Additional surgery of this sort must be balanced with risk of prolonged ischemic time. Heart transplantation is effective in Fontan patients with intractable arrhythmias, advanced heart failure, and protein-losing enteropathy.
Survival is worse compared to patients with other forms of congenital heart disease or with cardiomyopathy. Protein-losing enteropathy usually improves following transplantation [ ]. Actuarial survival without transplantation after a Fontan operation for patients born before was The principal causes of death were thromboembolic, heart failure related, and sudden death [ ]. Adult Fontan patients often live with low cardiac output, which drops further with atrial arrhythmias. Almost universal functional limitation is related to limited capacity for augmentation of cardiac output with activity.
Multi-system dysfunction related to high venous pressure is distressingly common, and includes hepatic dysfunction, [ - ] renal dysfunction [ ], endothelial dysfunction [ ], lower extremity venous insufficiency and venous reflux [ ], and coagulation abnormalities [ ]. Fontan conversion surgery can be carried out with relatively low mortality 5. The causes of mortality in this series were multiple, and included intractable heart failure and coronary artery disease.
The overall arrhythmia recurrence rate was Although the Fontan operation provides good palliation to most patients with functionally one ventricle, it has serious longterm limitations. It seems likely that most, if not all, Fontan patients will become transplant candidates because of heart failure. Novel approaches are needed to this complex problem. He was the picture of health. Bob never considered that a heart attack could happen to him. Bob was working out, as he did each day, when he lost consciousness.
He had to be shocked three times by an automated external defibrillator AED device to restart his heart. Today, Bob is passionate about another kind of training: While changes to his diet and exercise routine are an important part of his physical recuperation, Bob credits further education with helping him evolve his mindset and believes this plays an equally important role. Heart attack survivors share their stories about how their heart attack impacted them.
Loved ones open up about the strength they see in their survivor. Life after a heart attack can be frightening for patients and their loved ones. Fortunately, the good news is that your healthcare team is with you every step of the way. This second chance begins the ongoing journey to heart health and well-being. Frequency of another heart attack in the United States. Risk factors that can affect your chance of another heart attack are your age, your medical history and other health conditions you may have.
The risk of a recurrent attack can be greatly reduced by quitting smoking, exercising and following a healthy diet. You are not alone. You and your healthcare team will work together to come up with the right care plan for you. Most patients have to adjust to life after a heart attack, including starting new medications to lower the chance of another heart attack or dying from one.
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