Giant aneurysm of the vertical vein in a case of supracardiac total anomalous pulmonary venous connection.

A 17-year-old female presented with mild cyanosis. Imaging showed supracardiac total anomalous pulmonary venous connections with a vertical vein travelling between the left pulmonary artery and the left bronchus with significant obstruction. There was a huge post-stenotic aneurysm of the vertical vein.

A classical case of the Gasul phenomenon.

This case demonstrates the development of secondary infundibular stenosis in a 10-year-old male child with documented large non-restrictive perimembranous ventricular septal defect in infancy - the classical Gasul phenomenon.

Late desaturation due to giant azygous vein 16 years after bidirectional cavopulmonary anastomosis.

We present a rare late manifestation of systemic venous collaterals in a 17-year-old female, 16 years after bidirectional cavopulmonary anastomosis, resulting in clinically unacceptable desaturation with progressive effort intolerance and cyanosis.

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.

Management of dyslipidemia in special groups.

Dyslipidemia management in situations like pregnancy, in diseases like rheumatoid arthritis, human immunodeficiency virus (HIV) disease, chronic liver disease, and in the elderly are challenging scenarios. Pregnancy is a contraindication for many drugs. The interaction of various drugs used in HIV infection and rheumatoid arthritis makes it even more difficult to treat with conventional and approved drugs for dyslipidemia. Elderly and chronic renal failure patients often do not tolerate the drugs very well and the data of dyslipidemia management is very different. Lastly, COVID-19 is a unique scenario where clear information is yet to be provided. In this manuscript, the current understanding and available data on the treatment of dyslipidemia in these special situations are discussed.

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.

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.

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.

Electronic health records and outpatient cardiovascular disease care delivery: Insights from the American College of Cardiology's PINNACLE India Quality Improvement Program (PIQIP).

BACKGROUND: There has been a push toward implementation of electronic health records (EHRs) in federally-funded hospitals under the current policies initiated by the Indian government, with a lack of evidence supporting their adoption. We analyzed data from the American College of Cardiology's PINNACLE (Practice Innovation and Clinical Excellence) India Quality Improvement Program (PIQIP) to evaluate the association between EHR use and quality of cardiovascular disease care in India. METHODS AND RESULTS: Between 2011-2016, we collected data on performance measures for patients with coronary artery disease (CAD), heart failure (HF) and atrial fibrillation (AF) among 17 participating practices in PIQIP. There were 19,035 patients with CAD, 9,373 patients with HF, and 1,127 patients with AF. Documentation of co-morbidity burden in patients with CAD was lower among practices with EHR-hypertension (49.8% vs. 52.1%, p=0.003), diabetes (34.9% vs. 38.3%, p<0.001), and hyperlipidemia (0.2 vs. 3.9%, p<0.001). On the contrary, documentation of medication prescription was higher in CAD patients seen at practices with EHR-aspirin (63.2% vs. 17.8%, p<0.001), clopidogrel (41.7% vs. 27.4%, p<0.001), beta-blockers (61.4% vs. 9.8%, p<0.001), and ACE-i or ARBs (53.9% vs. 16.4%, p<0.001). Similarly, documentation of receipt of beta-blockers (43.8% vs. 10.7%, p<0.001), ACE-i or ARBs (40.8% vs. 16.1%, p<0.001), and beta-blockers+ACE-i or ARBs (36.4% vs. 3.6%, p<0.001) was also significantly higher in patients with HF seen at practices with EHR. Among patients with AF, documentation of oral anticoagulation use was significantly higher among EHR practices-warfarin (42.5% vs. 26.1%, p<0.001). CONCLUSIONS: Documentation of receipt of guideline-directed medical therapy in CAD, HF, and AF was significantly higher in practices with EHRs in India compared with sites without EHRs. Our findings shed a spotlight on the value of EHRs in future health care policy-making in India with regard to widespread adoption of EHRs in primary and advanced specialty care settings across public and private sectors.

Modified transjugular approach for percutaneous atrial septal defect closure.

Femoral venous route is routinely used for percutaneous closure of atrial septal defects (ASDs). However, a situation may arise where transfemoral approach is not feasible. We describe a successful transjugular closure of a moderate-sized ASD in a 49-year-old symptomatic man with interrupted inferior vena cava, using a novel deployment technique, which helped in overcoming difficulties such as maintaining stable sheath position and minimizing risk of air embolism.

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.

Gender disparities in cardiovascular care access and delivery in India: Insights from the American College of Cardiology's PINNACLE India Quality Improvement Program (PIQIP).

BACKGROUND: Limited data are available to assess whether access to and quality of cardiovascular disease (CVD) care are comparable among men and women in India. We analyzed data from the American College of Cardiology's PINNACLE (Practice Innovation and Clinical Excellence) India Quality Improvement Program (PIQIP) to evaluate gender disparities in CVD care delivery. METHODS AND RESULTS: Between 2011 and 2015, we collected data on performance measures for patients with coronary artery disease (CAD) (n=14,010), heart failure (HF) (n=11,965) and atrial fibrillation (AF) (n=496) in PIQIP, among 17 participating practices. The total number of women was 31,796 (32.0%). Women had fewer total encounters compared to men during the study interval (mean number of encounters=2.59 vs. 2.82 for women and men, respectively, p≤0.001). Women were significantly younger (48.9years vs. 51.5years, p≤0.01), but had a higher co-morbidity burden compared to men - hypertension (62.0% vs. 45.6%, p≤0.01), diabetes (39.4% vs. 35%, p≤0.01), and hyperlipidemia (3.7% vs. 3.1%, p=0.19). On the contrary, the guideline-directed medication prescriptions were strikingly lower in women with CAD compared to men - aspirin (38% vs. 50.4%, p≤0.001), aspirin or thienopyridine combination (46.9% vs. 57.2%, p≤0.001), and beta-blockers (36.8% vs. 47.8%, p≤0.001). Similarly, among women with ejection fraction ≤40%, the use of guideline-directed medical therapy was significantly lower compared to men for beta-blockers (30.8% vs. 37.0%, p≤0.001), angiotensin-converting enzyme inhibitors (ACE-i) or angiotensin receptor blockers (ARBs) (29.3% vs. 34.9%, p≤0.001), and beta-blockers/ACE-i or ARBs (24.6% vs. 31.0%, p≤0.001). Among patients with atrial fibrillation and CHADS2 score≥2, more women were on oral anticoagulation (19.6% vs. 14.6%, p=0.34), although this was not significantly different, and the overall number of patients with atrial fibrillation was low. CONCLUSIONS: Despite a significantly higher co-morbidity burden in women, we found fewer women receiving guideline-directed medical therapy for CVD compared with men. If such disparities are confirmed in the larger Indian population, it is important to find potential causes for, and seek solutions to narrow this gap.

American College of Cardiology (ACC)'s PINNACLE India Quality Improvement Program (PIQIP)-Inception, progress and future direction: A report from the PIQIP Investigators.

Cardiovascular diseases have surpassed infectious disorders to become the leading cause of morbidity and mortality in India.1 A national-level registry comprehensively documenting the current-day prevalence of cardiovascular risk factors and disease burden among patients seeking care in the outpatient setting in India is currently non-existent. With a burgeoning urban population, the cardiovascular disease burden in India is set to skyrocket, with an estimated 18 million productive years of life lost by 2030.2 While there are limited quality improvement registries in India, for example, the Kerala acute coronary syndrome and Trivandrum heart failure registries, their focus is on in-patient care quality improvement, while the vast majority of patients with cardiovascular diseases worldwide, including India, interact with the health care system in the outpatient setting.3,4 Recognizing this unmet need, the American College of Cardiology partnered with local stakeholders in India to establish India's first outpatient cardiovascular disease performance measurement initiative in 2011, the PINNACLE (Practice Innovation and Clinical Excellence) India Quality Improvement Program (PIQIP).5 This manuscript discusses the inception of the PIQIP registry, the progress it has made and challenges thus far, and its future direction and the promise it holds for cardiovascular care quality improvement in India.