Challenges of management and therapy in patients with a functionally single ventricle after Fontan operation

Forty years ago, Fontan and Baudet performed the first life-saving operation on a patient with a functionally single ventricle. This multi-stage procedure established the connection between systemic venous circulation and pulmonary arteries. As a consequence, the pulmonary circulation is supplied in a passive way, whereas the single ventricle pumps the blood into the systemic circulation only. Over the years, the technique of creating the abovementioned vascular connections has undergone several modifications. Due to the fundamental non-physiological hemodynamic relations between arterial pulmonary and systemic venous pressures, numerous complications can be observed in these patients including: supraventricular arrhythmias, thromboemboli, hepatic dysfunction, protein-losing enteropathy, heart failure, worsening cyanosis, systemic venous collateralization, and pulmonary arteriovenous malformations, as well as connective tissue lesions in bronchi. Although based on an ingenious concept, the operation remains of a palliative character. Occasionally, heart transplantation is the ultimate resolution. Pharmacological therapy, and surgical conversion, often appear to be ineffective. However, this procedure has enabled many patients to reach adulthood and enjoy their lives to the full. This fact poses a great challenge for cardiologists wishing to become more knowledgeable and experienced as regards such patients, if we are not to waste such fabulous surgical achievements. (Cardiol J 2011; 18, 2: 119-127

Arterial hypertension and organo-vascular pathological changes as late complications after coarctation of the aorta repair

Leczenie chirurgiczne jednej z częstszych wad wrodzonych serca - koarktacji aorty - jest możliwe od ponad pół wieku, coraz więcej chorych poddaje się postępowaniu inwazyjnemu. Nie powoduje ono jednak nigdy całkowitego wyleczenia. Zabiegi na tętnicy głównej pozostawiają bowiem zawsze pewne rezydualne zwężenie, a u niektórych chorych przyczyniają się do jej aneuryzmatycznego poszerzenia. Często współistniejąca z koarktacją dwupłatkowa zastawka aortalna ulega nieuchronnej degeneracji. Ponadto współczesne badania dowodzą, że wada ta nie jest jedynie prostą mechaniczną przeszkodą w przepływie krwi przez aortę zstępującą, lecz uogólnioną chorobą naczyniową. Z tego względu nawet po tak zwanej "skutecznej" korekcji wady obserwuje się systematyczny postęp choroby i wynikające z niego powikłania narządowe, do których przyczyniają się głównie nadciśnienie tętnicze oraz wczesne zmiany miażdżycowe, a długość życia chorych po operacji koarktacji aorty jest istotnie krótsza niż przeciętnej populacji. Dotychczas nie poznano w pełni patogenezy nadciśnienia tętniczego w analizowanej populacji. Obowiązują dzisiaj dwie zasadnicze teorie: mechaniczna, związana z obecnością zwężenia aorty zstępującej i zmniejszonej podatności aorty wstępującej, oraz neurohormonalna, zakładająca zmienioną reaktywność baroreceptorów i nawiązująca do zjawiska Goldblatta - hiperreaktywności układu renina-angiotensyna-aldosteron w wyniku względnej hipoperfuzji nerek. Współcześnie nie dysponujemy jednoznacznymi rekomendacjami dotyczącymi leczenia farmakologicznego nadciśnienia w tej grupie chorych, wiadomo natomiast że pacjenci po operacji koarktacji aorty powinni znajdować się pod stałą kontrolą kardiologów wyspecjalizowanych w wadach wrodzonych serca. Wskazane jest ścisłe monitorowanie kliniczne, niektórzy chorzy wymagają bowiem ponownego zabiegu. Systematyczny rozwój technik zabiegowych, a także poszerzanie wiedzy o późnych powikłaniach poprawia rokowanie tej grupy chorych. Nadciśnienie Tętnicze 2010, tom 14, nr 6, strony 480-489Surgical treatment of one of the most often found congenital heart diseases - coarctation of the aorta is possible since over half of the century and more and more patients are the subject to such invasive management. However, such procedure does not lead to complete recovery due to the fact that surgery on the main artery always leaves some residual narrowing and in some cases may be the cause of its aneurismatic dilatation. What is more, bicuspid aortic valve often coexisting with coarctation of the aorta is the subject of inevitable degeneration. Results from the recent studies prove that coarctation of the aorta is not only the problem of simple mechanic obstruction for the blood flow in the descending aorta but a more generalized vascular disease. On this account, even after so called "successful" correction of the coarctation, the consistent progression of the disease is observed with development of consequent target organ complications arising mainly from following arterial hypertension and early atherosclerotic changes. As a result the span of life of such patients is significantly shorter than in the general population. Pathogenesis of arterial hypertension in the analyzed population is still not fully understood. Currently there are two main theories concerning this subject: mechanistic, explaining the development and progression of the disease by obstruction of the descending aorta and decreased compliance of the precoarctation region of the main artery, and neurohormonal one, assuming changed reactivity of baroreceptors and referring to Goldblatt phenomenon - hyperreactivity of the rennin-angiotensin-aldosteron system as a result of relative kidneys’ hypoperfusion. Up to the date we have no unequivocal recommendations concerning pharmacological management of arterial hypertension in this group of patients, but it is obvious and unquestionable that subjects after surgical correction of the aortic coarctation should be under constant and close supervision of cardiologists specialized and experienced in management of congenital heart diseases. Systematic clinical monitoring of such patients is indicated as some of them may require re-operation in the future. Ongoing development of surgical techniques and widening the knowledge on late complications gradually improves the clinical outcome of those patients. Arterial Hypertension 2010, vol. 14, no 6, pages 480-48

Clinical factors affecting survival in patients with D-transposition of the great arteries after atrial switch repair: A meta-analysis

BACKGROUND: Atrial switch repair (AtrSR) was the initial method of operation in patients with D-transposition of the great arteries (D-TGA) constituting the right ventricle as a systemic one. Currently, it has been replaced with arterial switch operation (ASO), but the cohort of adults after AtrSR is still large and requires strict cardiological management of late complications. For this reason, we aimed to evaluate potential long-term mortality risk factors in patients with D-TGA after AtrSR (either Mustard or Senning procedure) METHOD: We searched MEDLINE database for suitable trials. We included 22 retrospective and prospective cohort studies of patients with D-TGA with at least 5 years mean/median follow-up time after Mustard or Senning procedure, with an end-point of non-sudden cardiac death (n-SCD) and sudden cardiac death (SCD) after at least 30 days after surgery. RESULTS: A total of 2912 patients were enrolled, of which 351 met the combined endpoint of n-SCD/SCD. The long-term mortality risk factors were: New York Heart Association class ≥ III /heart failure hospitalization (OR = 7.25; 95% CI, 2.67–19.7), tricuspid valve regurgitation (OR = 4.64; 95% CI, 1.95–11.05), Mustard procedure (OR = 2.15; 95% CI, 1.37–3.35), complex D-TGA (OR = 2.41; 95% CI, 1.31–4.43); and right ventricle dysfunction (OR = 1.94; 95% CI, 0.99–3.79) tends to be a risk factor. Supraventricular arrhythmia (SVT; OR = 2.07; 95% CI, 0.88–4.85) and pacemaker implantation (OR = 2.37; 95% CI, 0.48–11.69) did not affect long-term survival in this group of patients. In an additional analysis, SVT showed a statistically significant impact on SCD (OR = 2.74; 95% CI, 1.36–5.53) but not on n-SCD (OR = 1.5; 95% CI, 0.37–6.0). CONCLUSIONS: This meta-analysis identified that at least moderate tricuspid valve regurgitation, NYHA class ≥ III / heart failure hospitalization, right ventricle dysfunction, complex D-TGA and Mustard procedure as risk factors of long-term mortality in patients after AtrSR

Out-of-hospital cardiac arrest: Do we have to perform coronary angiography?

Out-of-hospital cardiac arrest (OHCA) remains a leading cause of global mortality, while survivors are burdened with long-term neurological and cardiovascular complications. OHCA management at the hospital level remains challenging, due to heterogeneity of OHCA presentation, the critical status of OHCA patients reaching the return of spontaneous circulation (ROSC), and the demands of post ROSC treatment. The validity and optimal timing for coronary angiography is one important, yet not fully defined, component of OHCA management. Guidelines state clear recommendations for coronary angiography in OHCA patients with shockable rhythms, cardiogenic shock, or in patients with ST-segment elevation observed in electrocardiography after ROSC. However, there is no established consensus on the angiographic management in other clinical settings. While coronary angiography may accelerate the diagnostic and therapeutic process (provided OHCA was a consequence of coronary artery disease), it might come at the cost of impaired post-resuscitation care quality due to postponing of intensive care management. The aim of the current statement paper is to discuss clinical strategies for the management of OHCA including the stratification to invasive procedures and the rationale behind the risk-benefit ratio of coronary angiography, especially with patients in critical condition