Anomalous origin of coronary artery from the opposite aortic sinus of Valsalva-a single center experience with a therapeutic conundrum.

BACKGROUND: Coronary artery anomalies are rare congenital abnormalities, most often found incidentally on conventional coronary angiography and CT angiography (CTA). CTA better delineates the origin and course of anomalous coronaries. Anomalous origin of coronary artery from the opposite aortic sinus of Valsalva (ACAOS) has a prevalence of 1% with a very few having an interarterial (malignant) course. There is limited literature, especially in the Indian population, dealing with this topic. METHODS: In this retrospective observational study, angiographic data of 8500 consecutive patients from June 2011 to December 2019 at a large tertiary care hospital in western India was analyzed. Patients diagnosed with ACAOS underwent CTA for delineation of the exact anatomy. Those with a non-malignant course with evidence of ischemia clinically or on stress myocardial perfusion imaging (MPI), underwent PCI. Others with a non-malignant course were medically managed. Patients with malignant (interarterial) course were revascularized by coronary artery bypass graft (CABG) surgery in case of LCA involvement or positive MPI test. Asymptomatic patients with negative MPI were managed medically. Clinical follow-up over 12 months of patients undergoing PCI and those with a malignant course showed no major adverse cardiovascular events (MACE). RESULTS: Of the 8500 patients studied, 74 (0.87%) had ACAOS. Of these, 51 (68.9%) patients had anomalous origin of right coronary artery (RCA) from the left aortic sinus, 21 (28.4%) had anomalous origin of the circumflex artery (Cx) from the right aortic sinus and two patients (2.7%) had an anomalous origin of the left main coronary artery (LCA) from the right aortic sinus. Interarterial course was found in five (6.7%) patients. Of these, four patients underwent CABG-one asymptomatic patient with LCA from right aortic sinus and three with positive MPI in anomalous RCA. All five patients with malignant course and ten patients who underwent PCI were free of MACE over 12 months' clinical follow up. CONCLUSIONS: ACAOS is a rare anomaly and if interarterial course is excluded, then PCI is feasible in selected cases with significant stenosis. Patients with malignant course with inducible ischemia or LCA involvement should undergo surgical revascularisation.

A longitudinal study of antibody responses to selected host antigens in rheumatic fever and rheumatic heart disease.

Introduction. Group A streptococci can trigger autoimmune responses that lead to acute rheumatic fever (ARF) and rheumatic heart disease (RHD).Gap Statement. Some autoantibodies generated in ARF/RHD target antigens in the S2 subfragment region of cardiac myosin. However, little is known about the kinetics of these antibodies during the disease process.Aim. To determine the antibody responses over time in patients and healthy controls against host tissue proteins - cardiac myosin and peptides from its S2 subfragment, tropomyosin, laminin and keratin.Methodology. We used enzyme-linked immunosorbent assays (ELISA) to determine antibody responses in: (1) healthy controls; (2) patients with streptococcal pharyngitis; (3) patients with ARF with carditis and (4) patients with RHD on penicillin prophylaxis.Results. We observed significantly higher antibody responses against extracellular proteins - laminin and keratin in pharyngitis group, patients with ARF and patients with RHD when compared to healthy controls. The antibody responses against intracellular proteins - cardiac myosin and tropomyosin were elevated only in the group of patients with ARF with active carditis. While the reactivity to S2 peptides S2-1-3, 8-11, 14, 16-18, 21-22 and 32 was higher in patients with ARF, the reactivity in the RHD group was high only against S2-1, 9, 11, 12 when compared to healthy controls. The reactivity against S2 peptides reduced as the disease condition stabilized in the ARF group whereas the reactivity remained unaltered in the RHD group. By contrast antibodies against laminin and keratin persisted in patients with RHD.Conclusion. Our findings of antibody responses against host proteins support the multistep hypothesis in the development of rheumatic carditis. The differential kinetics of serum antibody responses against S2 peptides may have potential use as markers of ongoing cardiac damage that can be used to monitor patients with ARF/RHD.

A rare endocrine cause of electrical storm - a case report.

Sheehan's syndrome, also called Simmond's syndrome, postpartum apoplexy, postpartum pituitary necrosis, and postpartum panhypopituitary syndrome, is the name given to postpartum hypopituitarism. The syndrome is caused by an infarction in the adenohypophysis, usually precipitated by massive uterine haemorrhage and hypovolemic shock during or after childbirth. Extensive destruction of pituitary cells results in varying degree of hypopituitarism. Acute loss of adenohypophysis function can be fatal without glucocorticoid and thyroid replacement therapy and survivors will require life time hormonal replacement therapy. Most cases present in the postpartum period with lactation failure or after months to years after the delivery. In many affected women, anterior pituitary dysfunction is not diagnosed for many years. Dyselectrolytemia is one of a common presentation in Sheehan's syndrome. Herein, we report a case of a 35-year old female with a history of obstetric hysterectomy 2 years ago in view of severe postpartum haemorrhage with history of failure of lactation and three episodes of syncope since last 1½ years and now presented with polymorphic ventricular tachycardia which required DC cardioversion. She was referred as a case of long QT syndrome. On investigating further, she had hypokalaemia secondary to hypopituitarism due to Sheehan's syndrome.

Cardiovascular Disease Performance Measures in the Outpatient Setting in India: Insights From the American College of Cardiology's PINNACLE India Quality Improvement Program (PIQIP).

BACKGROUND: India has a growing burden of cardiovascular disease (CVD), yet data on the quality of outpatient care for patients with coronary artery disease, heart failure, and atrial fibrillation in India are very limited. We collected data on performance measures for 68 196 unique patients from 10 Indian cardiology outpatient departments from January 1, 2011, to February 5, 2014, in the American College of Cardiology's PINNACLE (Practice Innovation and Clinical Excellence) India Quality Improvement Program (PIQIP). PIQIP is India's first national outpatient CVD quality-improvement program. METHODS AND RESULTS: In the PIQIP registry, we estimated the prevalence of CVD risk factors (hypertension, diabetes, dyslipidemia, and current tobacco use) and CVD among outpatients. We examined adherence with performance measures established by the American College of Cardiology, the American Heart Association, and the American Medical Association Physician Consortium for Performance Improvement for coronary artery disease, heart failure, and atrial fibrillation. There were a total of 68 196 patients (155 953 patient encounters), with a mean age of 50.6 years (SD 18.2 years). Hypertension was present in 29.7% of patients, followed by diabetes (14.9%), current tobacco use (7.6%), and dyslipidemia (6.5%). Coronary artery disease was present in 14.8%, heart failure was noted in 4.0%, and atrial fibrillation was present in 0.5% of patients. Among eligible patients, the reported use of medications was as follows: aspirin in 48.6%, clopidogrel in 37.1%, and statin-based lipid-lowering therapy in 50.6% of patients with coronary artery disease; RAAS (renin-angiotensin-aldosterone system) antagonist in 61.9% and beta-blockers in 58.1% of patients with heart failure; and oral anticoagulants in 37.0% of patients with atrial fibrillation. CONCLUSIONS: This pilot study, initiated to improve outpatient CVD care in India, presents our preliminary results and barriers to data collection and demonstrates that such an initiative is feasible in a resource-limited environment. In addition, we attempted to outline areas for further improvement in outpatient CVD care delivery in India.

Classical Cardiovascular Manifestations of Marfan Syndrome.

Presence of multiple cardiovascular manifestations of the Marfan syndrome in the same patient is not commonly encountered. We present a 49 year-old lady with this syndrome who presented with decompensated heart failure. Evaluation revealed presence of extensive Stanford type A aortic dissection alongwith severe aortic and mitral incompetence. However, the patient declined surgery and was discharged on medical management. At a year's follow-up, she had dyspnea of NYHA class II with persistent cardiovascular findings.

Simultaneous transcatheter closure of ruptured sinus of Valsalva aneurysm and stent implantation for aortic coarctation.

Ruptured sinus of Valsalva aneurysm is a rare anomaly and an associated coarctation of aorta is even rarer. A combination of such defects is traditionally treated surgically. The surgery is necessarily staged and done through different approaches. We report successful simultaneous transcatheter treatment of both these defects performed in the same setting in an acutely ill adult male patient with a good intermediate-term follow-up.

Supracardiac total anomalous pulmonary venous connection with bilateral (right and left) vertical veins and bilateral obstruction.

This report describes a rare form of obstructed supracardiac total anomalous pulmonary venous connection with two vertical veins (right and left) draining the right- and the left-sided pulmonary veins respectively without formation of a common chamber and with bilateral obstruction. Surgery for these patients is technically challenging due to the absence of a common chamber, and the prognosis is worse than for patients with a common chamber. Also, it is important to be aware of this entity so that an accurate noninvasive preoperative diagnosis by echocardiography alone without invasive cardiac catheterization is possible. This facilitates emergency surgical repair without delay, which is crucial to improvement of the outcome.

Transcatheter closure of ruptured sinus of Valsalva aneurysm using the Amplatzer duct occluder: immediate results and mid-term follow-up.

AIMS: To assess the immediate and mid-term outcome of transcatheter closure (TCC) using the first-generation Amplatzer duct occluder (ADO) in patients with ruptured sinus of Valsalva aneurysm (SOVA). Ruptured SOVA is a rare cardiac shunt lesion, with scant data about its TCC. METHODS AND RESULTS: Twenty patients (8 females and 12 males) aged 17-52 years (median 27 years) with ruptured SOVA were selected for TCC. Most (13/20) were in symptomatic NYHA class III or IV. Three had previous cardiac surgeries. Associated defects were bicuspid aortic valve in one, trivial pre-existing aortic regurgitation (AR) in five, coarctation of the aorta in one, and secundum atrial septal defect in one. Patients with co-existing ventricular septal defect or significant AR requiring surgery were excluded. Echocardiography revealed ruptured SOVA from right coronary sinus to right atrium (RA) in 4 and right ventricular (RV) outflow in 5, whereas non-coronary sinus ruptured into RA in 10 and RV inflow in 1. At cardiac catheterization, the defect was 4-11 mm (median 9 mm) at its aortic end as measured by online transoesophageal echocardiography or angiography. The Q(p)/Q(s) ratio ranged from 1.5 to 3.2 (mean 2.32 ± 0.53). In all patients, the defect was closed from the venous side, using ADOs 2-4 mm larger than the aortic end of the defect. The ADO sizes ranged from 8/6 to 16/14 mm (median 13/11 mm). The procedure was successful in 18 out of 20 patients (90%). Of these 18, 13 had a complete closure at discharge. Five had a residual shunt (four small and one moderate with self-abating haemolysis). Trivial AR occurred in four. On a median follow-up of 24 months (range 1-60 months), 15 patients were in NYHA class I and 3 in class II. The residual shunt disappeared in three and was small in two; procedure-related AR vanished in two of four. There was no AR progression, recurrence, infective endocarditis, or device embolization. CONCLUSION: In appropriately selected patients with ruptured SOVA, TCC is an attractive alternative to surgery with encouraging short- and mid-term outcomes.