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1.
Pediatr Cardiol ; 2024 Apr 18.
Article in English | MEDLINE | ID: mdl-38635040

ABSTRACT

Closure of the large ventricular septal defects (VSD) in infancy can lead to normalization of growth, but data are limited. Our study is done to assess the growth pattern in different age groups of children and lower birth weight babies after shunt closure. This is a prospective observational study that included infants with isolated large VSD operated in infancy. Anthropometric data were collected at baseline and at follow-up, and growth patterns were analyzed. 99 infants were included in the study. The mean age and weight at the time of surgery were 6.97 ± 2.79 months and 5.07 ± 1.16 kg, respectively. The mean follow-up duration was 8.99 ± 2.31 months. The weight for age (W/A) was the most adversely affected parameter preoperatively, and there was significant improvement noted in the mean Z score for W/A after shunt closure (- 3.67 ± 1.18 vs. - 1.76 ± 1.14, p = 0.0012). There was improvement in Z-scores for length for age (L/A) and weight for length (W/L), although it was not statistically significant. The infants from all the age groups had statistically significant growth in the anthropometric parameters. The rate of weight gain was maximum in the infants operated below 8 months of age (2-4 months = 3588 g, 5-6 months = 3592 g, 7-8 months = 3606 g, 9-10 months = 2590 g, 11-12 months = 2250 g). Low birth weight and normal birth weight infants had similar Z-scores at the time of surgery and at follow-up in all 3 anthropometric parameters, and birth weight did not affect pre- as well as post-operative growth parameters. Suboptimal improvement in weight and length was seen in 40 and 20% of babies even after successful surgical repair, respectively. Growth failure in infants with a large VSD can be multifactorial. Early surgical closure of the shunt can lead to early normalization of growth parameters and faster catch-up growth. Few babies may fail to demonstrate a positive growth response even after timely surgical correction, and may be related to intrauterine and genetic factors or faulty feeding habits.

2.
Cardiol Young ; 34(2): 436-438, 2024 Feb.
Article in English | MEDLINE | ID: mdl-38084422

ABSTRACT

Abernethy malformation is a congenital extra-hepatic porto-systemic shunt. This malformation is characterized by an abnormal connection between the portal vein or its branches and one of the systemic veins. Though rare, this anomaly can lead to pulmonary hypertension. Drainage of Abernethy malformation into coronary sinus is extremely rare. We describe a child with Abernethy malformation with unusual drainage into coronary sinus. The abnormal channel was successfully closed by trans-catheter technique with normalisation of pulmonary arterial pressures.


Subject(s)
Hypertension, Pulmonary , Vascular Malformations , Child , Humans , Hypertension, Pulmonary/complications , Portal Vein/surgery , Portal Vein/abnormalities , Catheters , Vascular Malformations/complications , Vascular Malformations/diagnosis , Vascular Malformations/surgery
3.
Indian J Thorac Cardiovasc Surg ; 40(1): 103-106, 2024 Jan.
Article in English | MEDLINE | ID: mdl-38125334

ABSTRACT

Aortic pseudoaneurysm is a rare but life-threatening complication after transcatheter closure of patent ductus arteriosus (PDA) with only a few reported cases in literature. We report a case of an 18-month-old child who was diagnosed to have pseudoaneurysm of the aorta after 6 months of transcatheter closure of PDA. It was diagnosed on routine follow-up on echocardiography evaluation. Computed tomography chest is the imaging modality of choice for evaluation of these patients which showed large saccular aneurysm arising from distal aortic arch beyond origin of left subclavian artery. Any aneurysm more than 30 mm should be treated irrespective of symptoms. Treatment modalities are surgical resection of the aneurysm (as in our patient) or thoracic endovascular aortic repair. In our case, surgical resection of aneurysm was performed. Supplementary Information: The online version contains supplementary material available at 10.1007/s12055-023-01619-z.

4.
Ann Pediatr Cardiol ; 16(2): 87-93, 2023.
Article in English | MEDLINE | ID: mdl-37767163

ABSTRACT

Introduction: To study the clinical presentation, laboratory profile, echocardiographic details, management, and outcomes of children who were diagnosed to have multisystem inflammatory syndrome in children (MIS-C) in the immediate postoperative period after surgery for congenital heart defects (CHDs). Materials and Methods: This is a prospective case-control study that included children diagnosed to have MIS-C in the postoperative period based on clinical signs, rise in inflammatory markers, and echocardiographic features of ventricular dysfunction or coronary involvement. Management included intravenous immunoglobulin (IVIG), steroids, and antiplatelet medications in addition to routine postoperative care. Out of the 461 children who underwent surgery for CHD between April 1st, 2021, and November 30th, 2021, 18 children were diagnosed with MIS-C. After the initial routine postoperative course, all 18 children had sudden worsening in clinical and laboratory parameters. Other causes such as bacterial infection were ruled out. All of these children had features of MIS-C with ventricular dilatation and dysfunction, coronary artery involvement, and reactive COVID-19 immunoglobulin G antibody. There was a significant improvement in coronary artery dimensions after IVIG administration (P = 0.001). The involvement of the left main coronary artery was associated with significantly increased length of intensive care unit (ICU) and hospital stay (P = 0.019). Mean ICU and hospital stay was prolonged in the MIS-C group. There were two deaths in this group due to severe left ventricular dysfunction. Conclusions: During the pandemic, a proportion of patients undergoing elective cardiac surgery may develop unexpected worsening in clinical status due to MIS-C. A high index of suspicion and prompt treatment with IVIG and steroids may be helpful in improving outcomes.

5.
Cardiol Young ; 33(3): 473-475, 2023 Mar.
Article in English | MEDLINE | ID: mdl-36647705

ABSTRACT

A common arterial trunk with pulmonary dominance is rare. We report the use of 3D volume-rendered CT imaging to define common arterial trunk with pulmonary arterial dominance and associated abnormalities.


Subject(s)
Truncus Arteriosus, Persistent , Humans , Pulmonary Artery/diagnostic imaging , Echocardiography , Tomography, X-Ray Computed , Aorta, Thoracic/diagnostic imaging
6.
Geriatr Nurs ; 50: 31-37, 2023.
Article in English | MEDLINE | ID: mdl-36640516

ABSTRACT

BACKGROUND: The study evaluated the effectiveness of a video-assisted exercise intervention program on fall incidence, activities of daily living, and fear of falling in community-dwelling older adults. METHODS: A video-assisted 16-week exercise intervention consisting of stretching, strengthening, balance, and dual-task training was delivered to randomly selected 95 older adults with a high risk of falls. The fidelity of implementation was assessed for three areas; exercise program delivery, participant receipt, and enactment. RESULTS: The fall incidence reduced significantly by 45% (IRR 0.55, (95% CI, 0.13-0.86) and difficulty in daily activities decreased (OR: 0.74, 95% CI, 0.16 - 0.96) among participants in the intervention group. Fear of falls did not show any significant difference in the groups at the 12-month follow-up. CONCLUSION: The video-assisted exercise program was found to be effective in reducing fall incidence among older adults at a higher risk of falls.


Subject(s)
Accidental Falls , COVID-19 , Aged , Humans , Accidental Falls/prevention & control , Activities of Daily Living , COVID-19/prevention & control , Exercise Therapy , Fear , Incidence , Independent Living , Postural Balance
7.
Cardiol Young ; 33(5): 780-786, 2023 May.
Article in English | MEDLINE | ID: mdl-35684953

ABSTRACT

BACKGROUND: Surgical care for CHD is increasingly available in low- and middle-income countries, and efforts to optimise outcomes are growing. This study characterises cardiac imaging and prenatal diagnosis infrastructure in this setting. METHODS: An infrastructure survey was administered to sites participating in the International Quality Improvement Collaborative for CHD. Questions regarding transthoracic, transesophageal and epicardial echocardiography, cardiac CT, cardiac magnetic resonance, prenatal screening and fetal echocardiography were included. Associations with in-hospital and 30-day mortality were assessed. RESULTS: Thirty-seven sites in 17 countries responded. Programme size and geography varied considerably: < 250 cases (n = 13), 250-500 cases (n = 9), > 500 cases (n = 15); Americas (n = 13), Asia (n = 18), and Eastern Europe (n = 6). All had access to transthoracic echo. Most reported transesophageal and epicardial echocardiography availability (86 and 89%, respectively). Most (81%) had cardiac CT, but only 54% had cardiac magnetic resonance. A third reported impediments to imaging, including lack of portable machines, age/size-appropriate equipment and advanced cardiac imaging access and training. Only 19% of centres reported universal prenatal CHD screening in their catchment area, and only 46% always performed fetal echocardiography if screening raised concern for CHD. No statistically significant associations were identified between imaging modality availability and surgical outcomes. CONCLUSIONS: Although access to echocardiography is available in most middle-income countries; advanced imaging modalities (cardiac CT and magnetic resonance) are not always accessible. Prenatal screening for CHD is low, and availability of fetal echocardiography is limited. Imaging infrastructure in low- and middle-income countries and associations with outcomes merits additional study.


Subject(s)
Developing Countries , Heart Defects, Congenital , Female , Pregnancy , Humans , Tomography, X-Ray Computed , Cardiac Imaging Techniques , Echocardiography , Ultrasonography, Prenatal
8.
Ann Pediatr Cardiol ; 16(4): 242-246, 2023.
Article in English | MEDLINE | ID: mdl-38343511

ABSTRACT

Background: Transcatheter patent ductus arteriosus (PDA) closure with unusual morphology associated with multiple constrictions or unusual circumstances such as interruption of inferior vena cava (IVC) is still challenging. This study evaluates the use of KONAR-MF™ (Lifetech Scientific Co Ltd., Shenzhen) for transcatheter closure of PDA in such situations. Materials and Methods and Results: Between January 2021 and October 2021, 24 patients from three different tertiary care centers underwent PDA device closure using the KONAR-MF™ device and are included in the study. Patient demographics, echocardiographic assessment, procedural details including the approach, device details, complications, and follow-up data were recorded. The median age and weight of the cohort were 2 years (0.5-41) and 10 kg (5-98), respectively. The indications for using KONAR-MF™ were (a) unusual morphology of PDA in 14, long tubular PDA in 8, and long tubular with multiple constrictions in 6 patients and (b) unusual circumstances such as interrupted IVC in 6 and inability to cross PDA antegradely in 4 due to dilated main pulmonary artery. The procedure was successful in all the patients and no procedure-related complications were noted in this cohort. Median follow-up of 9 months (range: 5-18) showed no residual shunt and only one patient had flow acceleration across the left pulmonary artery (peak Doppler gradient 16 mmHg). Conclusions: KONAR-MF™ device can be used safely and effectively for transcatheter closure of PDA with unusual anatomy or in unusual circumstances.

9.
Ann Pediatr Cardiol ; 15(2): 114-120, 2022.
Article in English | MEDLINE | ID: mdl-36246745

ABSTRACT

Background: Congenital portosystemic shunts (CPSS) are rare and present variably with hepatic encephalopathy, pulmonary arteriovenous malformations (PAVMs), and pulmonary hypertension (PH). Objective: The objective of the study was to see the feasibility of transcatheter closure of CPSS and their outcome. Materials and Methods: We analyzed the data of 24 patients of CPSS who underwent transcatheter closure from five institutions (March 2013 to April 2019). Baseline evaluation included echocardiography with bubble contrast study, ultrasound examination of the abdomen, computed tomography angiogram, and cardiac catheterization with test balloon occlusion of the CPSS. The evaluation showed cyanosis due to PAVM in 12, PH in 8, and respiratory distress in 2. Two had both cyanosis and PH. Criteria for eligibility for complete catheter closure of CPSS included demonstration of intrahepatic portal vein (PV) radicals together with a PV pressure of ≤18 mmHg on occlusion. Results: The median age and weight were 8 years (0.5-21) and 19.5 kg (4.2-73), respectively. Transcatheter closure was performed in 21 patients (22 procedures) using a variety of occlusive devices and stent-graft exclusion was done in one patient. Closure was not done in 3 in view of high portal venous pressures and hypoplastic PVs. During the follow-up (median: 42 months and range: 61 days-4.8 years), saturation normalized in 14 patients with PAVM. PH declined in all eight patients who underwent the procedure. Respiratory distress improved in two patients. Conclusions: Early and short-term follow-up results of catheter closure of CPSS appear promising. However, further, follow-up is needed to demonstrate long-term effectiveness.

10.
BMC Geriatr ; 22(1): 203, 2022 03 14.
Article in English | MEDLINE | ID: mdl-35287596

ABSTRACT

BACKGROUND: Declining functionality affects an individual's musculoskeletal integrity increasing the risk of fall and disability. Individuals with severe functional limitations are 5 times more likely to experience a fall. Thus, this paper investigated the association between functional decline and falls in older adults. METHODS: This study uses secondary data from the Longitudinal Aging Study in India (2017-18). A total of 31,477 people over the age of 60 are included in the study. Descriptive statistics and bivariate analysis were performed to determine the association between activities of daily living (ADL), instrumental activities of daily living (IADL) and fall. Adjusted odds ratio was used to determine the association of ADL and IADL with fall while controlling for age, gender, balance and gait impairments. RESULTS: The study reported 6352 fall episodes in 3270 participants aged 60 and above, over a period of 2 years. More than 30% of participants reported difficulty in ADL and IADL. Age and gender adjusted odds of fall were higher in participants with difficulty in more than 4 ADLs (AOR:1.32; CI:1.08 - 1.67) and in more than 2 IADL (AOR: 1.39; CI:1.02 - 1.89). Similarly, the odds of fall were higher for difficulty in ADL (AOR:1.31; CI:1.11 - 1.73) and IADL (AOR of 1.18; CI:1.07 - 1.29) controlling for gait and balance impairment. Difficulty in pushing-pulling objects (AOR: 1.30; CI: 1.15 - 1.46 & AOR: 1.40; CI: 1.21-1.61) and getting up from the chair (AOR: 1.12; CI:1.01-1.26 & AOR: 1.27; CI: 0.99 - 1.26) was significantly associated with fall when adjusted for age, gender and balance and gait parameters. CONCLUSIONS: This study provides the new insights into the association of fall and risk of functional decline, especially difficulty in pushing and pulling objects and getting up from a chair, can be incorporated in the primary screening of fall risk assessment.


Subject(s)
Activities of Daily Living , Disabled Persons , Aged , Aging , Humans , India/epidemiology , Longitudinal Studies
11.
Ann Pediatr Cardiol ; 15(5-6): 467-488, 2022.
Article in English | MEDLINE | ID: mdl-37152503

ABSTRACT

Justification: In recent years, there has been increasing recognition of children with heart disease in our country. These children belong to different age groups and have untreated, partially treated, or completely treated heart disease. The role of physical activity for optimal physical, emotional, and psychosocial well-being for children is well understood. There is a challenge for the parents and the medical professionals to take a decision regarding the type of physical activity safe for the child as heart disease may affect the hemodynamic demands. Most of the existing international guidelines focus on competitive sports in operated heart disease children. This may be of limited use when we have a mixed population of children with heart disease, different types of sports in our country and where a larger subset is looking for recommendations to leisure time activities. Process: The Pediatric Cardiac Society of India decided to formulate recommendations for physical activity in children with heart diseases. A committee of experts, who were well-versed with the subject of physical activity in children with heart disease, volunteered to take up the task of writing the guidelines. The recommendations emerged following deliberations of the committee members, on the virtual platform as well as mails. The final version of manuscript was approved by all committee members and all members are co-authors of this manuscript. The different types of physical activities were defined including leisure sports and competitive sports. The exercise was classified based on the mechanical action of muscles involved into dynamic and static components. Each type of exercise was then classified based on the intensity into low, medium, and high. Recommendations for the type of physical activity for individual heart lesions were decided based on the rationale available. Objectives: The recommendations here are made with an intention to provide general guidelines for physical activity in children with operated and unoperated heart diseases, not excluding a need for individualizing a plan, serial assessment, and comprehensive checkup in special situations. Recommendations: We hope the recommendations mentioned below would provide basic clarity in planning physical activity in children with heart disease. This is with the hope to encourage physically active life, at the same time ensuring a safety net.

12.
Natl Med J India ; 35(6): 338-343, 2022.
Article in English | MEDLINE | ID: mdl-37167510

ABSTRACT

Background Pulmonary arterial hypertension (PAH) is a progressive disease with high morbidity and mortality. Risk stratification and initiation of dual or triple combination therapy has a better clinical response, especially in high-risk patients. Unfortunately, prostacyclin analogues are not marketed in India; hence, the use of these medications is limited. We report the benefits and difficulties of using iloprost inhalation in patients with advanced PAH in India. Methods In this prospective observational study, we included patients with group 1 PAH. Inhaled iloprost was initiated as an add-on therapy for patients who had clinical, echocardiographic or laboratory deterioration on dual oral medications. Patients with clinical instability were excluded. All patients underwent thorough clinical evaluation, detailed echocardiogram and laboratory investigations. Patients were started on inhaled iloprost 2.5 µg six times daily and closely followed up. The dose was escalated if necessary. On follow-up, clinical echocardiographic and laboratory evaluation was done on all patients. Results Fourteen patients (11 women) with a median age of 32 years (2-66 years) with group 1 PAH were started on inhaled iloprost as an add-on therapy. Improvement in clinical parameters, WHO functional class, echocardiographic-derived right ventricular function, and N-terminal pro-brain natriuretic peptide (NT-pro-BNP) levels were observed in 10 of 14 patients. A median increase of 31% (4.2, 106%) in the distance travelled during 6-minute walk test, a median increase of 45% (-20, 120%) in right ventricular fractional area change, a median increase of 27% (-16.7, 60%) in tricuspid annular peak systolic excursion and a median decrease of 36.7% (-69.6, 17.2%) in NT-pro-BNP levels were observed after initiation of medication. Three patients had progression of symptoms and were then referred for lung/heart-lung transplant. One patient developed progression of symptoms after an excellent initial response and transitioned to subcutaneous treprostinil. Improvement in clinical, echocardiographic and laboratory features allowed us to successfully perform surgical Potts shunt in 2 patients. The medications were well tolerated with minimal and transient side-effects. There were no deaths. Conclusion Inhaled iloprost can be used with acceptable benefits and minimal side-effects in patients with PAH.


Subject(s)
Hypertension, Pulmonary , Pulmonary Arterial Hypertension , Humans , Female , Adult , Iloprost/therapeutic use , Iloprost/adverse effects , Vasodilator Agents/adverse effects , Hypertension, Pulmonary/drug therapy , Hypertension, Pulmonary/diagnosis , Pulmonary Arterial Hypertension/chemically induced , Pulmonary Arterial Hypertension/drug therapy , Administration, Inhalation
13.
Ann Pediatr Cardiol ; 14(3): 260-268, 2021.
Article in English | MEDLINE | ID: mdl-34667395

ABSTRACT

BACKGROUND: COVID-19 pandemic has disrupted pediatric cardiac services across the globe. Limited data are available on the impact of COVID.19 on pediatric cardiac care in India. AIMS: The aims are to study the impact of COVID-19 pandemic on the care of children with heart disease in India in terms of number of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries. SETTINGS AND DESIGN: This is a retrospective, multicentric, observational study. METHODS: We collected monthly data on the number and characteristics of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries and major hospital statistics, over a period of 5 months (April to August 2020), which coincided with the first wave of COVID-19 pandemic in India and compared it with data from the corresponding months in 2019. RESULTS: The outpatient visits across the 24 participating pediatric cardiac centers decreased by 74.5% in 2020 (n = 13,878) as compared to the corresponding period in 2019 (n = 54,213). The reduction in the number of hospitalizations, cardiac surgeries, and catheterization procedures was 66.8%, 73.0%, and 74.3%, respectively. The reduction in hospitalization was relatively less pronounced among neonates as compared to infants/children (47.6% vs. 70.1% reduction) and for emergency surgeries as compared to elective indications (27.8% vs. 79.2%). The overall in-hospital mortality was higher in 2020 (8.1%) as compared to 2019 (4.8%), with a higher postoperative mortality (9.1% vs. 4.3%). CONCLUSIONS: The current COVID-19 pandemic significantly impacted the delivery of pediatric cardiac care across India with two-third reduction in hospitalizations and cardiac surgeries. In an already resource-constrained environment, the impact of such a massive reduction in the number of surgeries could be significant over the coming years. These findings may prove useful in formulating strategy to manage subsequent waves of ongoing COVID-19 pandemic.

14.
Ann Pediatr Cardiol ; 14(3): 269-277, 2021.
Article in English | MEDLINE | ID: mdl-34667396

ABSTRACT

BACKGROUND: Outcome data of children with heart disease who acquired COVID-19 infection are limited. AIMS: We sought to analyze outcome data and identify risk factors associated with mortality in children with heart disease and grown-ups with congenital heart disease (GUCH) who had a laboratory-confirmed COVID-19 infection. SETTINGS AND DESIGN: This is a retrospective, multicentric, observational study. MATERIALS AND METHODS: The study included children with heart disease and GUCH population, who presented with either symptomatic or asymptomatic COVID-19 infection to any of the participating centers. COVID-19-negative patients admitted to these centers constituted the control group. RESULTS: From 24 pediatric cardiac centers across India, we included 94 patients with a median age of 12.5 (interquartile range 3-96) months and 49 (52.1%) patients were males. Majority (83 patients, 88.3%) were children. One-third of the patients (n = 31, 33.0%) had acyanotic congenital heart disease, and 41.5% (n = 39) were cyanotic, with > 80% of the patients being unoperated. Only 30 (31.9%) patients were symptomatic for COVID-19 infection, while the rest were incidentally detected positive on screening. A total of 13 patients died (case fatality rate: 13.8%). The in-hospital mortality rate among hospitalized patients was significantly higher among COVID-19-positive cases (13 of 48; 27.1%) as compared to COVID-negative admissions (9.2%) during the study period (P < 0.001). On multivariate analysis, the independent predictors of mortality among COVID-19-positive cases were severity of illness at admission (odds ratio [OR]: 535.7, 95% confidence interval [CI]: 6.9-41,605, P = 0.005) and lower socioeconomic class (OR: 29.5, 95% CI: 1.1-814.7, P = 0.046). CONCLUSIONS: Children with heart disease are at a higher risk of death when they acquire COVID-19 infection. Systematic preventive measures and management strategies are needed for improving the outcomes.

15.
Indian J Thorac Cardiovasc Surg ; 37(5): 569-572, 2021 Sep.
Article in English | MEDLINE | ID: mdl-34511766

ABSTRACT

Cor triatriatum sinister is a rare congenital cardiac anomaly. It consists of a fibro-muscular membrane that separates the left atrium into two chambers resulting in a tri-atrial heart. It is often found in association with other structural cardiac anomalies. The "acquired" form is extremely rare and only anecdotal reports are found in literature. We describe a case of acquired cor triatriatum following surgical repair of coronary sinus type of total anomalous pulmonary venous connection (TAPVC), which presented 11 years after initial repair of TAPVC.

16.
Gait Posture ; 88: 16-21, 2021 07.
Article in English | MEDLINE | ID: mdl-33951574

ABSTRACT

BACKGROUND: An unstable gait pattern is an indicator of an increased risk of falls among older adults. Data on basic gait parameters is useful in the early identification of gait impairment. However, reference gait measurements are not available in low- and middle-income countries. RESEARCH QUESTION: What are the normative reference values of gait parameters and do fall risk factors such as impaired balance, functional difficulty, and multimorbidity affect the gait patterns of older adults in India? METHODS: A cross-sectional data of 659 older adults were collected using a semi-structured schedule. Gait parameters were measured using wearable sensors. Descriptive statistics, independent t-test, and one-way ANCOVA were used to determine the significant difference (p < 0.05) in gait parameters across the risk factors. RESULTS: A mean stride length of 123.00 ±â€¯15.19 cm, stride velocity of 110.57 ±â€¯17.57 cm/s, and a cadence of 106.14 ±â€¯11.44 steps/minute were reported in the study. Functional difficulties and balance impairment were the two major risk factors that affected stride velocity, stride length, and cadence after adjusting for age and height. No difference in gait parameters was observed among participants with and without multimorbidity. SIGNIFICANCE: This study provides a baseline or reference values of various gait parameters measured on a large sample of population aged 60 and above from India. Assessment of gait patterns and associated risk factors in a clinical setup will help identify the older adults at risk of falls and reduce the enormous burden of fall injuries. Since gait parameters show a large variation across geographical regions, it is important to have region-specific reference values.


Subject(s)
Accidental Falls , Gait , Aged , Cross-Sectional Studies , Humans , Reference Values , Risk Factors
17.
Indian Heart J ; 73(2): 196-204, 2021.
Article in English | MEDLINE | ID: mdl-33865518

ABSTRACT

BACKGROUND: Potts shunt has been suggested as an effective palliative therapy for patients with pulmonary artery hypertension (PAH) not associated with congenital heart disease. MATERIALS AND METHODS: This is a prospective single-center study performed to assess outcomes of Potts shunt in patients with PAH who are in functional class III or IV. RESULTS: 52 patients in functional class III/IV with pulmonary arterial hypertension without significant intra or extracardiac shunt on maximal medical therapy were evaluated and counseled for undergoing Potts shunt/patent ductus arteriosus (PDA) stenting. 16/52 patients (13 females) consented for the procedure; 14 patients underwent surgical creation of Potts, and 2 underwent transcatheter stenting of PDA, which physiologically acted like a Potts shunt. Standard medical therapy was continued in patients who did not consent for the procedure. 12/16 patients survived the procedure. Patients who did not survive the procedure were older, with severe right ventricular systolic dysfunction, and functional class IV. Patients who survived the procedure were followed up in the pulmonary hypertension clinic. The Median follow-up was 17 months (1-40 months). 11/13 patients discharged after the operation showed sustained clinical, echocardiographic, and biochemical improvement, which reduced need for pulmonary vasodilator therapy in 10/13 patients. There was one death in the follow-up period 16 months post-surgery due to lower respiratory tract infection. CONCLUSION: Potts shunt is feasible in patients with PAH without significant intra or extracardiac shunts. It can be done safely with an acceptable success rate. Patient selection, preoperative stabilization, and meticulous postoperative management are essential. It should be performed at the earliest sign of clinical, echocardiographic, or laboratory deterioation for optimal outcomes. Long-term follow-up is required to see a sustained improvement in functional class and the need for a lung transplant in the future.


Subject(s)
Pulmonary Arterial Hypertension , Pulmonary Artery , Anastomosis, Surgical , Female , Humans , Palliative Care , Prospective Studies , Pulmonary Artery/surgery , Treatment Outcome
18.
Indian Heart J ; 73(1): 99-103, 2021.
Article in English | MEDLINE | ID: mdl-33714417

ABSTRACT

OBJECTIVE: Abernethy malformation (congenital extrahepatic portosystemic shunt) is a rare anomaly of the splanchnic venous system. Though rare, it is an important cause of pulmonary artery hypertension (PAH) which is often missed. All patients with PAH should be carefully evaluated for presence of Abernethy malformation before labelling them as Idiopathic PAH. METHODS: This is a retrospective analysis of prospectively collected data. We reviewed the data of all patients referred to our center for evaluation of PAH. 10 patients were diagnosed to have an extrahepatic portocaval malformation. We reviewed their presentation, diagnosis, catheterization data, intervention and their outcome along with review of literature. RESULTS: 10/104 patients with pulmonary hypertension and no intra or extracardiac shunt were found to have extrahepatic portocaval shunt (EHPCS). 3 patients had EHPCS type 1 and 7 had type 2 EHPCS. 6/7 patient with EHPCS type 2 underwent closure of the shunt. There was no procedure related complication. There was one death 3 months post procedure and one patient who was advised surgical closure was lost to follow up. Closure of the shunt resulted in normalization of the pulmonary artery pressures in 4/5 patients. CONCLUSION: Congenital portosystemic malformations form an important and potentially treatable cause of pulmonary hypertension.


Subject(s)
Hypertension, Pulmonary/etiology , Portal Vein/abnormalities , Vascular Malformations/complications , Vena Cava, Inferior/abnormalities , Adolescent , Adult , Angiography , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Hypertension, Pulmonary/diagnosis , Infant , Male , Portal Vein/diagnostic imaging , Rare Diseases , Retrospective Studies , Vascular Malformations/diagnosis , Vena Cava, Inferior/diagnostic imaging , Young Adult
20.
Indian Pediatr ; 57(11): 1015-1019, 2020 11 15.
Article in English | MEDLINE | ID: mdl-32788432

ABSTRACT

OBJECTIVE: We describe the presentation, treatment and outcome of children with multisystem inflammatory syndrome with COVID-19 (MIS-C) in Mumbai metropolitan area in India. METHOD: This is an observational study conducted at four tertiary hospitals in Mumbai. Parameters including demographics, symptomatology, laboratory markers, medications and outcome were obtained from patient hospital records and analyzed in patients treated for MIS-C (as per WHO criteria) from 1 May, 2020 to 15 July, 2020. RESULTS: 23 patients (11 males) with median (range) age of 7.2 (0.8-14) years were included. COVID-19 RT-PCR or antibody was positive in 39.1% and 30.4%, respectively; 34.8% had a positive contact. 65% patients presented in shock; these children had a higher age (P=0.05), and significantly higher incidence of myocarditis with elevated troponin, NT pro BNP and left ventri-cular dysfunction, along with significant neutrophilia and lympho-penia, as compared to those without shock. Coronary artery dilation was seen in 26% patients overall. Steroids were used most commonly for treatment (96%), usually along with intra-venous immunoglobulin (IVIg) (65%). Outcome was good with only one death. CONCLUSION: Initial data on MIS-C from India is presented. Further studies and longer surveillance of patients with MIS-C are required to improve our diagnostic, treatment and surveillance criteria.


Subject(s)
COVID-19/diagnosis , Systemic Inflammatory Response Syndrome/diagnosis , Adolescent , Biomarkers/blood , COVID-19/epidemiology , COVID-19/therapy , Child , Child, Preschool , Female , Glucocorticoids/therapeutic use , Humans , Immunoglobulins, Intravenous/therapeutic use , India/epidemiology , Infant , Lymphopenia/etiology , Male , Myocarditis/etiology , Natriuretic Peptide, Brain/blood , Neutrophils/metabolism , Peptide Fragments/blood , Shock/etiology , Systemic Inflammatory Response Syndrome/epidemiology , Systemic Inflammatory Response Syndrome/therapy , Troponin/blood , Ventricular Dysfunction, Left/etiology
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