Pathophysiology of Congenital Heart Disease in the Adult

Sommer, Robert J.; Hijazi, Ziyad M.; Rhodes, John F.

doi:10.1161/circulationaha.107.714428

Cited by 64 publications

(40 citation statements)

References 65 publications

(51 reference statements)

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“…In the symptomatic patients, tricuspid valvuloplasty or valve replacement provide satisfactory hemodynamic improvement. In selected patients with right ventricular cardiomyopathy [23], one-and-a-half ventricular repair is an alternative with satisfactory results. However, in the presence of pulsatile pulmonary flow, some patients with one-and-a-half ventricular circulation may have a certain degree of superior vena cava syndrome and need to be closely monitored [26].…”

Section: Discussionmentioning

confidence: 99%

Clinical spectrum and long-term outcome of Ebstein’s anomaly based on a 26-year experience in an Asian cohort

et al. 2008

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Ebstein's anomaly is a rare, congenital cardiac anomaly that may result in cyanosis, right heart failure, and tachyarrhythmia during the newborn stage or after adolescence. This study investigated the data of 77 patients diagnosed between 1980 and 2006 at a tertiary care center in Taiwan. Patients were grouped into either an early group or a late group. Survival declined rapidly within the newborn stage in the early group, but declined only during the third decade in the late group. Surgical results were poor (20% success rate) for neonatal systemic-to-pulmonary shunts in those cases with associated pulmonary atresia, but were satisfactory for other surgical modes. Supraventricular tachyarrhythmia occurred in 31 (41%) patients at a median age of 10 years and could be eliminated by radiofrequency ablation (81% success rate), though the recurrence rate was high (41%). In conclusion, other than those cases requiring shunts at the newborn stage, the long-term outcome was favorable.

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Section: Discussionmentioning

confidence: 99%

Clinical spectrum and long-term outcome of Ebstein’s anomaly based on a 26-year experience in an Asian cohort

et al. 2008

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“…Tetralogy of Fallot (TOF) is one of the most common types of cyanotic heart lesions and occurs in 8% to 9.7% of children with congenital heart disease [1,2]. TOF is a conoventricular abnormality that consists of four anatomical malformations: (1) a ventricular septal defect; (2) a pulmonary arterial stenosis; (3) an aortic valve that overrides the interventricular septum; and (4) hypertrophy of the right ventricle, which may not occur until the neonatal period [3,4].…”

Section: Introductionmentioning

confidence: 99%

Hypoxia induces PGC-1α expression and mitochondrial biogenesis in the myocardium of TOF patients

et al. 2010

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PGC-1α, a potent transcriptional coactivator, is the major regulator of mitochondrial biogenesis and activity in the cardiac muscle. The dysregulation of PGC-1α and its target genes has been reported to be associated with congenital and acquired heart diseases. By examining myocardium samples from patients with Tetralogy of Fallot, we show here that PGC-1α expression levels are markedly increased in patients compared with healthy controls and positively correlated with the severity of cyanosis. Furthermore, hypoxia significantly induced the expression of PGC-1α and mitochondrial biogenesis in cultured cardiac myocytes. Mechanistic studies suggest that hypoxia-induced PGC-1α expression is regulated through the AMPK signaling pathway. Together, our data indicate that hypoxia can stimulate the expression of PGC-1α and mitochondrial biogenesis in the cardiac myocytes, and this process might provide a potential adaptive mechanism for cardiac myocytes to increase ATP output and minimize hypoxic damage to the heart.

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“…This abnormality is most often congenital 1 and, although representing the most common coronary arterial malformation, is a rare cardiac anomaly. The incidence is of 0.002% of the general population and of 0.4% of all cardiac malformations.…”

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confidence: 99%

An unusual case of ST elevation myocardial infarction in a teenager with a coronary artery fistula.

Cortese

Gesualdo

Acquaviva³

et al. 2016

ICFJ

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Coronary artery fistula (CAF) is an abnormal connection between a coronary artery and any of the 4 chambers of the heart or any of the great vessels (superior vena cava, pulmonary artery, pulmonary veins, or coronary sinus). This abnormality is most often congenital 1 and, although representing the most common coronary arterial malformation, is a rare cardiac anomaly. The incidence is of 0.002% of the general population and of 0.4% of all cardiac malformations. 2Fistulas originating from the right coronary artery account for 50% to 60% of cases, while those from the left anterior descending artery for 25% to 42% of cases, 18.3% from the circumflex artery, 1.9% from the diagonal branch, and 0.7% from the left main coronary artery or circumflex-marginal branch. 3,4 The most common are single fistulas, with a prevalence ranging from 74% to 90%, multiple fistulas occur in 10.7% to 16%, while both coronary arteries are involved in only 5%. 2The right heart is the most frequent site of drainage of fistulas 5 ; in particular, the pulmonary artery is the drainage site in 15% to 43% of cases, followed by the right ventricle in 14% to 40%, the right atrium in 19% to 26%, the left ventricle in 2% to 19%, the coronary sinus in 7%, the superior vena cava in 1% and finally the left atrium in 5% to 6%. 3,4 Moreover literature data show that bilateral fistulas, accounting for 5% of the total, terminate more often into the pulmonary artery (56%) than unilateral fistulas (17%). Pathological manifestations depend on the resistance of the connection and the fistula drainage site. The resistance is determined by the characteristics (size, tortuosity, and length) of the fistula. Flow through fistulas that drain in the right chambers occurs during the whole cardiac cycle loading both ventricles, while when the drain is in the left atrium and pulmonary vein, volume overload affects only the left heart. CAFs are usually asymptomatic at a young age, unless they are of large dimensions. Symptoms can occur with the increase of age: fatigue, dyspnea, palpitations and ischemic chest pain are the most frequent clinical manifestations whereas heart failure, pulmonary hypertension, subacute bacterial endocarditis, rupture or thrombosis the extreme complications. 6 We report a rare case of bilateral CAF draining into the left atrium determining myocardial infarction in an 18 year old boy. A young man of 18 years, with no cardiovascular risk factors and without family history of juvenile sudden death and ischemic heart disease was hospitalized for chest pain radiating to the left arm. Medical history showed right lobar pneumonia at the age of 3 years, and a diagnosis of myopericarditis at 14 years. The patient was not taking medication.At the first medical contact blood pressure was 130/80 mmHg, the general, cardiac and pulmonary examinations were in the normal range. The ECG showed sinus rhythm at the frequency of 68 bpm with ST-segment elevation from V3 to V6 and in D1, D2 and aVL, negative T waves in D3 and aVF. Chest X-ray showed enlargement ...

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Pathophysiology of Congenital Heart Disease in the Adult

Cited by 64 publications

References 65 publications

Clinical spectrum and long-term outcome of Ebstein’s anomaly based on a 26-year experience in an Asian cohort

Clinical spectrum and long-term outcome of Ebstein’s anomaly based on a 26-year experience in an Asian cohort

Hypoxia induces PGC-1α expression and mitochondrial biogenesis in the myocardium of TOF patients

An unusual case of ST elevation myocardial infarction in a teenager with a coronary artery fistula.

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