Shay Cullen

Pulmonary regurgitation is an important determinant of right ventricular contractile dysfunction in patients with surgically repaired tetralogy of fallot

Background—Evaluation of right ventricular (RV) function in patients with pulmonary regurgitation (PR) after tetralogy repair remains challenging because of abnormal RV loading conditions. Methods and Results—We examined 124 patients, aged 21±11.4 years, who had tetralogy repair at 3.7±3.5 years. By Doppler echocardiography, 33 patients had mild, 22 moderate, and 69 severe PR; 55 had significant tricuspid regurgitation (TR). Myocardial velocities, myocardial acceleration during isovolumic contraction (IVA), strain, and strain rate were measured at RV and LV base. Tricuspid valve annulus was measured in a 4-chamber view. QRS, QT, and JT intervals and their dispersions were measured from 12-lead electrocardiogram. IVA in the RV was lower in all patients compared with controls (0.8±0.4 versus 1.8±0.5, P<0.0001) and correlated with the severity of PR (r=−0.43, P<0.0001), whereas myocardial velocities, and strain/strain rate did not. LV IVA correlated with PR (r=−0.32, P<0.001) and with RV IVA (r=0.28, P<0.003). Patients with severe PR had a higher incidence of TR (r=0.69, P<0.0001) and lower RV IVA (1.0±0.4 versus 0.6±0.3, P<0.0001), a larger tricuspid valve ring diameter (P<0.0001), and prolonged electrical depolarization (P<0.001). Age at surgery or examination did not correlate with PR and with RV function assessed by IVA. In the RV, IVA correlated inversely with QRS duration (P<0.01). Conclusions—Although load-dependent myocardial velocities and strain are not influenced by the severity of PR and presence of significant TR, IVA demonstrates reduced contractile function in relation to the degree of PR and may be an early, sensitive index for selecting patients for valve replacement.

Quality of life of adult congenital heart disease patients: a systematic review of the literature

AIMS This review explores the quality of life of adult congenital heart disease patients and the relationship between disease severity and quality of life. METHODS We searched seven electronic databases and the bibliography of articles. The 31 selected studies fulfilled the following criteria: adult population; quantitative; assessment of quality of life and/or impact of disease severity on quality of life using validated measures; English language. Data extraction forms were used to summarise the results. RESULTS There are evident methodological limitations within the reviewed studies such as heterogeneous populations, designs, and quality of life conceptualisations and measurements. Despite these problems, findings suggest that the quality of life of adult congenital heart disease patients is compromised in the physical domain compared with their healthy counterparts, whereas no differences were found in relation to the psychosocial and environmental/occupational domain. Some severity variables appear to be significant correlates of quality of life and could be considered in a future standardised classification of disease severity. CONCLUSION The methodological limitations of past research in relation to the definition and measurement of quality of life, the study designs, and disease severity classifications need to be addressed in future studies in order to provide robust evidence and valid conclusions in this area of study. This will enable the development of targeted interventions for the improvement of quality of life in the adult population of congenital heart disease patients.

Cognitive dysfunction in adult CHD with different structural complexity.

OBJECTIVE We carried out a cross-sectional study to assess cognitive function in a sample of adult CHD patients, within the Functioning in Adult Congenital Heart Disease study London. The association between cognitive functioning and disease complexity was examined. METHODS A total of 310 patients participated in this study. Patients were classified into four structural complexity groups - tetralogy of Fallot, transposition of the great arteries, single ventricle, and simple conditions. Each patient underwent neuropsychological assessment to evaluate cognitive function, including memory and executive function, and completed questionnaires to assess depression and anxiety. RESULTS Among all, 41% of the sample showed impaired performance (>1.5 SD below the normative mean) on at least three tests of cognitive function compared with established normative data. This was higher than the 8% that was expected in a normal population. The sample exhibited significant deficits in divided attention, motor function, and executive functioning. There was a significant group difference in divided attention (F=5.01, p=0.002) and the mean total composite score (F=5.19, p=0.002) between different structural complexity groups, with the simple group displaying better cognitive function. CONCLUSION The results indicate that many adult CHD patients display impaired cognitive function relative to a healthy population, which differs in relation to disease complexity. These findings may have implications for clinical decision making in this group of patients during childhood. Possible mechanisms underlying these deficits and how they may be reduced or prevented are discussed; however, further work is needed to draw conclusive judgements.

The Surgical History, Management, and Outcomes of Subaortic Stenosis in Adults

BACKGROUND Subaortic stenosis (SAS) is a curtain of tissue involving the subaortic region, the aortic and mitral valves, the septum, and the fibrous trigones. Little is known of its course or the outcomes of its surgical management in adults. METHODS We reviewed our experience of the surgical management of SAS in adults from 1999 to 2010. We divided patients into three groups: (1) those presenting for first-time SAS resection (6 patients, 4 male, median age of 46.9 ± 17 years, mean follow-up of 5 ± 2.7 years); (2) those requiring redo resection of SAS without organic aortic valve dysfunction (8 patients, 3 male, median age of 25.3 ± 5 years, mean follow-up of 8 ± 3.08 years); and (3) those with SAS and aortic valve dysfunction (8 patients, 4 males, median age of 34.8 ± 12 years, mean follow-up of 4.5 ± 2.5 years; 5 had previous SAS surgery). RESULTS Patients underwent extensive excision of the SAS, release of the fibrous trigones, and a septal myectomy if required. There was 1 early death in group 2 and 1 in group 3. In group 3, 1 patient underwent the Ross procedure and 7 patients had mechanical valve implantation. No patient required permanent pacemaker implantation. Overall follow-up was 3.3 ± 3 years (range, 6 months to 10 years). The preoperative left ventricular outflow tract gradient ranged from 40 to 120 mm Hg, and the postoperative left ventricular outflow tract gradient ranged from 0 to 16 mm Hg. At latest follow-up, no patient in groups 1 or 2 had greater than mild native aortic regurgitation. CONCLUSIONS Subaortic stenosis resection in adults can successfully relieve left ventricular outflow tract obstruction, with low mortality. The complexity of SAS increases with time; therefore a longer duration of follow-up is needed to further validate our conclusions.

Extraction of Pacing Leads Jailed by a Stent in a Mustard Circulation

A case of successful lead extraction of atrial and ventricular pacing leads “jailed” by a stent in the superior limb of a Mustard baffle in a 40‐year‐old man with surgically corrected transposition of the great arteries and pacemaker pocket infection. Manual traction alone was sufficient to remove the jailed leads with no subsequent complications. (PACE 2010; e65–e67)

Postcardiotomy syndrome: recurrent cardiac tamponade and an exquisite steroid response

A 26-year-old woman presented moribund with fever and pleuritic chest pain 3 times in 4 months following elective aortic root surgery. She was admitted 41 days after surgery with cardiac tamponade requiring surgical drainage twice within 1 week. Despite this, she was re-admitted for a second time 4 days after discharge with persistent pericardial effusion. High fevers and an incidental regurgitant murmur were extensively investigated for and treated as possible endocarditis or graft infection without conclusive results. The patient spent a total of 61 days in hospital during this period, receiving seven different antibiotic courses. Her third admission, with most severe clinical features, nearly led to further surgery and removal of her aortic graft but instead culminated in a multidisciplinary team decision to initiate steroid therapy for postcardiotomy syndrome. A short course of oral prednisolone saw her pericardial effusion and symptoms resolve completely.